splenomegaly
Introduction
Introduction Splenomegaly is an important pathological sign. Under normal circumstances, the spleen is generally not touched. If the supine or lateral position can touch the edge of the spleen, the spleen should be considered enlarged. People with low or thin diaphragm muscles, especially women, can touch the edge of the spleen, but they are quite soft and have no tenderness, which is different from pathological splenomegaly. Increased spleen volume is a major manifestation of spleen disease.
Cause
Cause
Etiology classification
The etiology classification of splenomegaly can be classified into two categories: one is infectious splenomegaly; the other is non-infectious splenomegaly.
First, infectious
(1) Acute infection
Found in viral infections, rickettsial infections, bacterial infections. Spirochete infection, parasitic infection.
(2) Chronic infection
Found in chronic viral hepatitis, chronic schistosomiasis, chronic malaria, kala-azar, syphilis, etc.
Second, infectious
(1) congestion
Found in cirrhosis, chronic congestive right heart failure, chronic constrictive pericarditis or a large number of pericardial effusion Judd-Chiari syndrome, idiopathic non-sclerosing portal hypertension.
(2) Blood diseases
Found in various types of acute and chronic leukemia, erythroleukemia. Red blood disease, malignant lymphoma, malignant histiocytosis, idiopathic thrombocytopenic purpura, hemolytic anemia, polycythemia vera, myelofibrosis, multiple myeloma, systemic tissue mast cell disease, hypersplenism disease.
(3) connective tissue disease
Such as systemic lupus erythematosus, dermatomyositis, nodular polyarteritis, juvenile rheumatoid arthritis (Sill disease) Felty disease.
(4) Histiocytosis
Such as Le Yi Xue (letterer-siwe) disease, yellow lipoma disease, (Han Yi Xue Yi Ke) syndrome, eosinophilic granuloma.
(5) Lipid deposition
Such as high snow disease, Niemann a disease.
(6) Spleen tumor and spleen cyst
Primary malignant tumors of the spleen are rare, and malignant tumors that metastasize to the spleen are rare. The primary cancer is mostly located in the digestive tract. Spleen cysts are rare, substantial and pseudocysts. True cysts are divided into epidermal cysts, endothelial cysts (such as lymphatic cysts) and parasitic cysts. The pseudocysts are classified as hemorrhagic, serum or inflammatory.
mechanism
There are many causes of splenomegaly, and the mechanism of splenomegaly caused by each disease is also different. Sometimes the mechanism by which a disease causes splenomegaly may be multiple factors. The mechanism is summarized as follows:
First, cell infiltration
Spleen enlargement caused by cell infiltration is seen in: various inflammatory cell infiltration, eosinophil infiltration, leukemia cell infiltration, and various tumor cell infiltration. Inflammatory cell infiltration is more common in acute infectious diseases, often accompanied by obvious congestion of the spleen. Eosinophil infiltration is seen in eosinophilia and partial lipid deposition; splenomegaly caused by infiltration of various leukemia cells is most pronounced in chronic myelogenous leukemia. Followed by chronic lymphocytic leukemia; lymphocytic type is more obvious in acute leukemia, followed by acute myeloid leukemia and acute monocytic leukemia. Various tumor cell infiltration, malignant tumors of the spleen itself are rare, and malignant tumors originating in the lymphatic system, bone marrow and intestine can invade the spleen. Such as: lymphoma cells of malignant lymphoma spleen infiltration, which is more affected by Hodgkin's disease spleen, accounting for about 50%, occasionally simple type Hodgkin's disease, this disease can show spleen extravagance Infiltration of myeloma bone marrow cancer cells; malignant histiocytic invasion of malignant histiocytosis; intestinal malignant tumor metastasis to the spleen, splenomegaly can occur due to tumor cell infiltration.
Second, the spleen blood stasis
The spleen is the largest blood storage organ in the body. The spleen blood flow is blocked by various reasons, which can cause congestion and swelling of the spleen. Such as: cirrhosis portal hypertension / pulse and inferior vena cava thrombosis, tumor emboli, congenital or acquired vascular malformations; right heart failure caused by various causes, constrictive pericarditis or pericardial effusion can cause The spleen is swollen and swollen.
Third, extramedullary hematopoiesis
The spleen is a hematopoietic organ. In the case of myeloproliferative diseases, the spleen restores its hematopoietic function. Different degrees of extramedullary hematopoiesis lead to splenomegaly. Especially in the case of myelofibrosis, extramedullary hematopoiesis is most obvious, and splenomegaly is also obvious.
Fourth, tissue cell proliferation
Histiocytosis is caused by abnormal proliferation of tissue cells, involving many organs of the body, especially in the liver, spleen, lymph nodes, bone marrow, skin, thymus, etc., which can show obvious splenomegaly. Such as: Le Yi Xue disease, Han * Xue Yi Ke disease / chronic infectious diseases, kala-azar, connective tissue disease, fellty, Still disease, rheumatoid arthritis, etc. can cause spleen tissue cells to proliferate and spleen month.
Fifth, fibrous tissue hyperplasia
Due to the long-term chronic stimulation of long-term chronic congestion, chronic infection, cell infiltration and other causes, the abnormal proliferation of tissue cells causes a large proliferation of spleen fibrous tissue, which causes the spleen to enlarge and become hard.
6. Metabolic disorders
Due to a deficiency or dysfunction of lipid metabolism enzymes, lipid metabolism disorders are caused, and lipids are deposited in tissues to cause splenomegaly. For example, high snow disease is an autosomal recessive lipid-like metabolic disorder. It is because glucose brain enzymatic enzyme can not convert glucose dyslipid into galactose cerebral palsy, resulting in a large amount of brain sputum accumulation in tissue cells, and the chronic type shows spleen. Niemann-Pick disease is a mononuclear macrophage tissue that affects the internal organs of lipid metabolism disorders. This disease may be due to the lack of neurophospholipidase, so that the neurolipids are stored in the liver and spleen and the macrophages of the nervous system, resulting in a significant increase in the spleen.
Seventh, the spleen itself and the cystic dilatation of the spleen itself are rare tumors and cystic expansion, there are reports of primary spleen malignant lymphoma. Cystic dilatation is seen in dermoid cysts, lymphatic cysts, and parasitic cysts (cysticercosis). Pseudocysts such as: malaria, schistosomiasis, etc. may be due to bleeding, inflammation. Caused by serum and other reasons.
Examine
an examination
Related inspection
Spleen percussion spleen palpation of gastric sputum drum area blood routine anti-hepatitis C virus IgA antibody
Physical examination
Spleen enlargement mainly depends on palpation examination. When the palpation method is not diagnosed, the percussion method can be used to check whether the dullness of the spleen is enlarged or not. (Normal spleen dullness is between the 9th-11th rib of the left iliac midline; 4-7cm wide, no more than the front of the anterior line can be examined by ultrasound, X-ray, CT, etc., to determine the size and shape of the spleen. Palpation When the splenomegaly is found, pay attention to the degree and quality of the splenomegaly, and pay attention to the presence or absence of other accompanying signs.
(a) the extent of splenomegaly
The extent of splenomegaly is associated with disease.
1. Mild splenomegaly: When the inhalation is deep, the lower edge of the card is 2-3cm under the costal spleen. Can be seen in certain viral infections, bacterial infections, rickettsial infections, early schistosomiasis, congestive heart failure, cirrhosis, portal hypertension, Hodgkin's disease. Juvenile rheumatoid arthritis, systemic lupus erythematosus, tropical eosinophilia, idiopathic thrombocytopenic purpura.
2, moderate splenomegaly: the lower edge is 3cm below the costal margin to the flat umbilicus is moderately enlarged. Can be seen in acute myeloid leukemia, acute lymphocytic leukemia, chronic hemolytic anemia, infectious mononucleosis, scutellaria, spleen amyloidosis, malignant lymphoma, Niemann-Pick disease.
3, extreme splenomegaly: the lower edge beyond the umbilical level is extremely splenomegaly or called giant spleen. Can be seen in chronic myeloid leukemia, chronic malaria, advanced schistosomiasis, spotted disease, myelofibrosis, polycythemia vera, thalassemia, high snow disease.
(2) the quality of the spleen;
The splenomegaly caused by different causes may have different degrees of quality. Generally, the card is caused by acute infection. The splenomegaly caused by chronic infection and leukemia cell tumor is hard and the surface is uneven; the spleen of liver cirrhosis is moderately hard; the splenomegaly is due to congestion. The degree and duration of the congestion are different, the time is short, the congestion is light, the quality is soft, the time is long or the heavyness of the congestion is hard, but generally there is a feeling of fullness. Cystic swelling has a scalp that is not smooth. It must be pointed out that the degree and quality of splenomegaly are not only different due to different causes, but also due to the course of the disease. There are differences in treatment and individual responsiveness, and these factors should be noted when judging.
(three) accompanying signs
Different causes of splenomegaly have different accompanying signs.
1, anemia, bleeding or ecchymosis: seen in hematological splenomegaly, such as various types of leukemia, idiopathic thrombocytopenia, sexual purple epilepsy.
2, anemia, yellow disease: seen in hemolytic anemia, chronic viral hepatitis, cirrhosis, malignant histiocytosis, sepsis.
3, liver and lymph node enlargement: seen in malignant lymphoma, lymphocytic leukemia, connective tissue disease, donor mononucleosis, sarcoidosis and some infectious diseases.
4, liver disease face, liver palm and spider fatigue: seen in chronic viral hepatitis, cirrhosis.
5, various types of rash: more common in various infectious diseases, or infectious diseases. Such as typhoid, typhus, brucellosis, sepsis, subacute infective endocarditis.
6, edema and ascites: seen in chronic right heart failure, constrictive pericarditis, cirrhosis, portal hypertension, inferior vena cava obstruction.
7, dirty expansion: seen in a variety of heart disease caused by chronic heart failure, a large number of pericardial effusion caused by various reasons.
Laboratory inspection
Laboratory tests have important implications for the diagnosis of splenomegaly, including routine blood tests, platelet counts, reticulocyte counts, and eosinophil counts. Careful blood cell card examinations are useful for detecting pathological blood cells and other abnormal cells. Hemoglobin electrophoresis, erythrocyte fragility test, acid hemolysis, (Ham), direct anti-human globulin (Combs) blood parasites, protozoan examination, liver function tests, bone marrow examination or bone marrow biopsy. Serological antigen antibody examination; lymph node puncture or biopsy, splenectomy or biopsy, ascites routine examination. The above laboratory tests can be based on the medical history, preliminary diagnosis, and targeted selection. For certain diseases, the basis for determining the diagnosis can often be found.
Device inspection
The necessary instrumental examination has important diagnostic value for determining the cause of splenomegaly. Commonly used examination methods are old-style ultrasound examination and B-ultrasound. It has been screened, X-ray, CT, MRI, endoscopy, and inferior vena cava.
The diagnosis steps for splenomegaly are inseparable from the above aspects, but there must be a primary and secondary in the thinking process. First determine whether the spleen is swollen, the extent and quality of the spleen. The second step is to understand the accompanying symptoms and signs of spleen enlargement. It is usually done by understanding the medical history and physical examination; by understanding the medical history, the physical examination can make a preliminary diagnosis of the cause of the splenomegaly, and then selectively perform laboratory tests and necessary instrument examinations, and finally the splenomegaly. The reason for making a diagnosis.
Diagnosis
Differential diagnosis
1. Spleen enlargement and differentiation of other organs in the left upper abdomen
The clinically enlarged left lobe of the liver is sometimes misdiagnosed as splenomegaly. If the palpation is found to be connected to the right lobe of the liver, it may be hepatomegaly rather than splenomegaly. Splenomegaly must be distinguished from left renal enlargement and renal ptosis. The latter is less likely to move in the retroperitoneal space. In addition, because the inflatable intestine is located in front of the kidney, the percussion is drum sound, which can be distinguished from splenomegaly. In addition, the following points help to identify other lumps in the abdominal cavity:
1 The spleen is located under the left costal margin and moves up and down following the breath.
2 has a clear edge with one or two notches on the edge.
3 is more accessible in front of the abdominal wall.
4 The location of the splenomegaly is perturbation, and it is connected with the left lower chest and spleen voiced area. There is no gap between the spleen and the costal margin. It is difficult to insert the hand into the human body. 5 Subcutaneous injection of adrenaline can make the congestive splenomegaly short. Significantly reduced in time, but this method must be applied with caution.
Second, the cause identification
(1) Infectious splenomegaly
1, acute infection: acute infectious splenomegaly often manifested as mild splenomegaly, soft texture, common diseases are:
(1) Viral infection: When the virus is infected with splenomegaly, blood lymphocytes often appear in the course of the disease, especially in the case of infectious mononucleosis, and can also be seen in viral hepatitis and rubella.
(2) Rickettsia infection: typhus and tsutsugamushi are often accompanied by mild splenomegaly. They belong to infectious diseases and need to pay attention to epidemiological data when diagnosing. For the diagnosis of typhus, the external sputum reaction (Proteus OX. Agglutination reaction) is the most common application, and its serum agglutination titer is valuable at 1:160. If it rises weekly, it has a definite diagnostic value. The diagnostic test for tsutsugamushi disease (patient sera and Proteus OX strain agglutination test) has important reference value, 1:80 is meaningful, and can gradually increase with the course of disease.
(3) bacterial infection: typhoid typhoid, brucellosis, acute miliary tuberculosis, sepsis, splenomegaly, common. Typhoid, paratyphoid and brucellosis are often characterized by fever, leukopenia and relative lymphocyte height. The blood-like eosinophils around the typhoid fever are reduced or disappeared. The diagnosis of fat-to-foot typhoid fever is of great value. Generally, the "o" antibody titer is 1:80 or more, and the "H" antigen has a diagnostic value of 1:160 or more. Blood and bone marrow culture are the basis for determining the diagnosis. Brucellosis often has a history of exposure to cattle, sheep, and pigs, and Brucella agglutination experiments. The complement fixation test is measured once a week, and has a higher titration titer or a multiplier increase in titer. A positive result of culture such as bone marrow or pus is the basis for diagnosis.
Acute miliary tuberculosis chest X-ray examination without abnormal findings, often difficult to diagnose, blood anti-tuberculosis antibodies and TBDN examination may be helpful, in the diagnosis of difficulties, anti-tuberculosis test treatment, if effective, can confirm the diagnosis. The splenomegaly of sepsis is generally mildly swollen, soft in texture, individual splenomegaly is obvious, and the texture is hard. Inflammatory endocarditis with pain when the card is embolized. A positive result in blood culture is the basis for determining the diagnosis. Spleen abscess is a rare disease, which is often secondary to splenic vein thrombosis, sepsis and abdominal purulent infection, and there are also unidentified primary infections. The clinical symptoms are similar to sepsis. The spleen has obvious tenderness or fluctuating sensation. When there is inflammation around the card, the spleen area can hear the rubbing sound or touch the friction. It must be differentiated from the left leg abscess or the abdominal wall abscess. CT guided spleen puncture has great value in the diagnosis of spleen abscess.
(4) spirochete infection: leptospirosis, rat bite heat and regression heat often accompanied by mild splenomegaly. Epidemiological data is an important clue to diagnosis.
(5) Parasitic infection: Acute malaria that has not been treated with antimalarial drugs. After several episodes, most of them can reach the swollen spleen, which is slightly tough. In areas with high incidence of malaria, patients with fever and coma, combined with splenomegaly, must consider the possibility of cerebral malaria. Blood smears found malaria parasites, which is the most reliable basis for diagnosis. About 60% of acute schistosomiasis is accompanied by splenomegaly, epidemic fusion, acute fever with hepatosplenomegaly, bloody leukocytosis, and classification of eosinophils as clues for the diagnosis of this disease. The detection of schistosomiasis eggs in feces is the basis for diagnosis.
2, chronic infection: chronic infection of the splenomegaly, the degree and hardness, due to the etiology, disease course, treatment and individual reactivity are different. Splenomegaly is generally non- tender and hard. The more obvious the fibrous tissue proliferation, the harder the spleen texture. Chronic infectious diseases often associated with splenomegaly are:
(1) Chronic viral hepatitis: Chronic viral hepatitis with splenomegaly is more common, generally mild, no obvious tenderness, mostly moderate hardness. There is still splenomegaly during the recovery period of acute hepatitis, suggesting a chronic tendency in the course of the disease. The main basis for diagnosis is medical history, liver function tests and hepatitis serum markers. A serum marker test can determine the type of pathogen of viral hepatitis.
(2) Chronic schistosomiasis: Chronic schistosomiasis is often accompanied by varying degrees of splenomegaly. When developing into cirrhosis, the spleen may be extremely swollen and hard due to congestion. Patients often have both portal hypertension and liver damage, and often have varying degrees of hypersplenism. In the case of advanced schistosomiasis, it is difficult to find eggs in the feces. The diagnosis is mainly based on the history of epidemiology, the history of schistosomiasis in the past, the above clinical manifestations and the intradermal test of positive schistosomiasis antigens, and the diagnosis of eggs by rectal biopsy is more clear. .
(3) Chronic malaria: Chronic malaria can have extreme splenomegaly and a hard texture. The longer the general course of disease, the less complete the treatment, the more significant the splenomegaly, resulting in a large amount of fibrous tissue hyperplasia, the quality can be as hard as wood. Its diagnosis relies on peripheral blood to find Plasmodium, but it is more difficult, and bone marrow and spleen puncture may be helpful in diagnosis. If you have doubts, you can do regular antimalarial treatment, and you can confirm the diagnosis effectively.
(4) Black fever: more common in the Yellow River Basin and the northwest region, it has been basically eliminated after vigorous prevention and control. The splenomegaly is the main sign, and the 2-3 months after the disease can reach 5 cm below the costal margin. The late reaches the extreme and the texture becomes hard. Other symptoms include irregular fever, physical weakness, loss of appetite, hepatomegaly, leukopenia, and increased monocytes. The aldosterone test product combination test is helpful for diagnosis, and the diagnosis is based on bone marrow puncture and smear examination, and the black fever pathogen is found.
(5) syphilis: early or late syphilis may have splenomegaly, mostly mild. The diagnosis is mainly based on medical history and clinical manifestations of X-ray examination. The medical history is extremely important. It is necessary to ask in detail about the smelting tour, sexual history, blood test history, treatment history and maternal birth history. Because of the transmission of syphilis, you should also know the illness of your parents, brothers and sisters. Commonly used experimental tests have the significance of the serum Kang's Fahrenheit reaction for the diagnosis and treatment of various stages of syphilis and the discovery of recessive syphilis. However, it may be a biological false positive reaction to certain other diseases such as malaria and systemic lupus erythematosus. Serum responses in some patients with syphilis are also always negative. The spirochete fluorescent antibody adsorption test is highly specific and can avoid biological false positives. The diagnosis of treponema pallidum in the lesion has a diagnostic value.
(6) Sarcoidosis: It is not uncommon in China. Foreign literature 50%-60% involving the liver and spleen. The lymph nodes involved are 100%. The cause is still unclear. The course of the disease is slow, generally no obvious symptoms, no fever or slight heat. Sarcoidosis with nodular erythema, often with fever and joint swelling and pain, hilar lymphadenopathy and increased erythrocyte sedimentation rate, must be distinguished from tuberculosis. Angiotensin-converting enzyme assay increased in the active period of sarcoidosis, the positive rate reached 90%, and has a clear diagnostic value. Superficial lymph node biopsy, subcutaneous nodules or skin nodule biopsy have important diagnostic value.
(two) non-infectious splenomegaly
1, spleen blood stasis: simple blood stasis splenomegaly, after the removal of the cause of congestion, swollen spleen can be significantly retracted, but long-term chronic congestion can be due to proliferation of mononuclear-macrophage system and fibrous tissue proliferation Significantly retracted. There are several common diseases that cause splenomegaly:
(1) Liver cirrhosis: 70%-80% of cirrhosis with splenomegaly, mostly mild, the longer the course of disease, the more obvious the swelling. Its diagnosis is based on medical history, liver function tests, etiological diagnosis, and objective evidence of portal hypertension. Determining the diagnosis depends on a liver biopsy.
(2) Budd-Chiari syndrome: In recent years, after extensive screening of B-mode ultrasound, many hepatic congestive sclerosis and portal veins due to thrombosis or stenosis above the hepatic vein and hepatic vein inferior vena cava have been found. Hyperbaric symptoms, known as Budd-Chiari syndrome, may be associated with splenomegaly. The bed is similar to cirrhosis caused by other causes, but is characterized by scabbard and obvious varicose veins on the lateral chest wall. "B-mode ultrasound findings of thrombosis or vascular stenosis above the hepatic vein or hepatic vein inferior vena cava population may prompt diagnosis, and inferior vena cava angiography may confirm the diagnosis.
(3) idiopathic portal hypertension: an unexplained extrahepatic portal hypertension* bed manifested as splenomegaly, hypersplenism, esophageal varices with repeated upper gastrointestinal bleeding; The histological changes of hardening, liver function is generally normal, and there is no decompensation performance such as ascites, jaundice, and hepatic encephalopathy after hemorrhage. B-mode ultrasound shows portal vein dilatation, which is helpful for diagnosis.
(4) Chronic right heart failure: When valvular heart disease occurs in chronic right heart failure, the spleen often does not enlarge due to venous blood stasis, but spleen atrophy often occurs due to hypoxia, but when developing cardiogenic cirrhosis There are many splenomegaly. It is not difficult to have a clear history of heart disease and its signs.
(5) Chronic constriction. Already covered with inflammation: about 50% of cases of chronic constrictive pericarditis have splenomegaly. Atypical cases are often misdiagnosed, and careful physical examination and X-ray and echocardiography are often used to confirm the diagnosis. Right atrial catheterization is required for individual atypical cases.
(6) portal vein thrombosis: rare, whether acute or chronic type have obvious splenomegaly. Acute type is often secondary to splenectomy / venous surgery / intravenous infection or trauma. Chronic type is often secondary to cirrhosis, and secondly, due to tumor compression or erosion of the portal vein in liver cancer or other organs in the abdominal cavity.
(3) Blood diseases
1. Leukemia: All types of acute and chronic leukemia can have splenomegaly. Among them, splenomegaly caused by acute and chronic leukemia is most common in granulocyte type, followed by lymphocyte type and mononuclear cell type, and chronic monocytic leukemia is rare. The diagnosis of leukemia mainly depends on blood and bone marrow. Clinically, it is still accustomed to classify leukemia into two categories: one is acute lymphoblastic leukemia (the other is acute non-lymphocytic leukemia).
1 acute myeloid leukemia.
2 acute granulocyte-monocytic leukemia.
3 acute monocytic leukemia.
4 red leukemia.
5 megakaryocytic leukemia.
The above types are divided into many subtypes according to the degree of differentiation of leukemia cells, such as acute myeloid leukemia and detachment (M3a, M3b) acute granulocyte-monocytic leukemia and sub-M a, Mb acute monocytic leukemia Subdivided into M5a, M5b, ME and so on.
(1) Acute leukemia
Green tumor: Acute myeloid leukemia, if accompanied by eyeballs, is most likely a green tumor. It is a rare type of acute myeloid leukemia. Clinically rare. There are dozens of domestic reports, all under the age of 30, of which children account for half, more men than women. Clinical manifestations include tumor formation in addition to signs of acute myeloid leukemia. Tumors can occur in any organ and tissue, especially in the eye sockets, head and chest. The tumor sections were pale green. This green color gradually fades in the air and sunlight. If the tumor tissue is placed in glycerin, the original green luster can be maintained. The clinical manifestations, blood and bone marrow of green tumors are the same as those of acute myeloid leukemia. There are also a few blood and bone marrow images that are identical to acute mononuclear (Neiglian) leukemia. In addition, there are rare and limited types of green tumors. In the whole process of the disease, the tumor is limited to somewhere in the body, and there is no change in blood and bone marrow.
(2) Chronic leukemia
Compared with chronic myeloid leukemia (slow granule); accounting for 90.5% of all types of chronic leukemia, the age of onset is 3-85 years old, and the highest incidence rate is 24-4O years old. Clinical manifestations include fever, weakness, weight loss, and extreme splenomegaly. Extreme splenomegaly is an important sign of chronic myeloid leukemia. The total number of white blood cells in the surrounding blood is extremely increased (100-300) X10/L). Various types of granulocytes, including the lobulated nucleus, rod-shaped nucleus, late, young and young, and promyelocytes, and a small number of granulocytes, have absolute values for neutral, eosinophilic, and basophils. Increased, it is a so-called "various" phenomenon. Myeloid hyperplasia is marked or extremely active, mainly due to increased granulocyte system, and the classification counts like peripheral blood, but the level of maturity is low. Most cases can be diagnosed based on extreme splenomegaly and typical blood and bone marrow changes.
Chronic myeloid leukemia, the course of the disease is generally more than one year may occur acute changes. The following points suggest the possibility of early urgency:
1 fever of unknown cause.
2 obviously thin.
3 progressive anemia and tendency to major bleeding.
4 bone and joint pain.
5 use busulfan or deep radiation.
The treatment is ineffective or the curative effect is not obvious, and the spleen does not shrink but tends to increase. In the late stage of catastrophe, in addition to the above symptoms, there may be pain in the card area, superficial lymph node enlargement, cachexia and so on. Bone marrow and blood are important basis for determining rapid changes. When the granulocyte plus promyelocytes in the blood image account for 0. 20 or more of the total number of counts, and the granulocytes and promyelocytes in the bone marrow account for 0.25 or more, the diagnosis of acute changes can be established. The identification of acute granulocytic leukemia and acute myeloid leukemia is generally not difficult to achieve according to the extreme splenomegaly. When there is difficulty, you can do a chromosome check. If there is a ph chromosome, it supports slow-grain catastrophe. There is no differential significance in the case of Ph-negative chronic myeloid leukemia.
In chronic myeloid leukemia, blood and bone marrow granulosa cells are dominant, neutrophil alkaline phosphatase activity is reduced, and serum vitamin B. The increase in content, Ph color positive (positive rate 90%) can be distinguished from late polycythemia, myelofibrosis and leukemia-like reactions.
b. Chronic lymphocytic leukemia: the domestic incidence is low. The difference with chronic myeloid leukemia is that the splenomegaly is milder, and the superficial lymph node enlargement is more obvious. The old cell sorting count is dominated by small and medium lymphocytes, and only a few young lymphocytes are swelled; Some cases can survive for up to 25 years. Typical cases can be diagnosed based on superficial lymphadenopathy and changes in blood levels. A multiple smear or lymph node biopsy can be diagnosed in atypical cases.
(3) rare types of leukemia
a, eosinophilic leukemia: clinically more acute, similar to general acute leukemia, a small number of chronic, like chronic myelogenous leukemia. The total number of white blood cells can be normal or increased to above 200 X10/L. The mature eosinophils in blood smears account for about 0.60-0.85, and the eosinophilic mesenchymal cells are less, only 0.005-0.02, and even granulocytes can appear. Bone marrow can be divided into primitive cell type or mature cell type. The former has a significant increase in granulocytes, while the latter has mature eosinophils, and the original granulocytes can be normal or slightly increased. The diagnosis of this disease depends on eosinophilia caused by other causes. The typical case diagnosis is based on: 1 significant increase in eosinophils in blood and bone marrow, and even myogenic granulocytes; 2 eosinophil infiltration in all organs; 3 abnormal morphology of eosinophils, "myeloid" "More to see.
b, basophilic leukemia: very rare, only a few reports in the country. The spleen can be extremely swollen. Clinical manifestations of anemia, thrombocytopenia, leukocytosis up to (100 ~ 200) X10 / L, half of which are basophils. In chronic myeloid leukemia, basophilic granulocytosis is often present in the course of the disease, especially in the case of advanced cases or eclipses. There is no clear standard for the identification of the two.
c., hairy cell leukemia: is a rare type of special type of leukemia, which is chronic. 80% have splenomegaly, half of the performance is extremely swollen, and no obvious lymphadenopathy, more common in middle-aged men, the onset age is 22-80 years old, most 40-60 years old. Insidious onset, l / 4 patients with bleeding points, ecchymosis, with white blood cells or thrombocytopenia, 1/4 patients with no symptoms, only found splenomegaly during physical examination. The blood picture is often reduced by whole blood cells, and it is possible to measure the number of hairy cell leukemia cells (hair cells but bone marrow are more easily found. The morphology is similar to that of large lymphocytes, but there are hairy protrusions on the edges. The morphology and large lymphoids under the microscope Cells and monocytes are indistinguishable and can be determined by phase microscopy or electron microscopy. 90%-95% of patients have positive anti-tartaric acid phosphatase reaction in hair cells, which is highly supportive for the diagnosis of this disease. Bone marrow cell examination has diagnostic value, but there are 113 cases were extracted and can be diagnosed by bone marrow biopsy.
d, red blood disease and erythroleukemia: all belong to the category of leukemia, very rare in the country, a large number of nucleated red blood cells in the surrounding blood suggest the diagnosis of red blood disease; if nucleated red blood cells and immature granulocytes appear at the same time, it suggests the diagnosis of erythroleukemia .
The diagnosis of red blood disease is based on:
1 severe progressive anemia, anti-anemia treatment is ineffective, and may be associated with fever, hemorrhage, mild swelling of the liver and spleen and lymph nodes.
2 blood is severe or moderate anemia, leukopenia or increase, but no immature granulocytes, and there are nucleated red blood cells, platelets often decrease.
3 The bone marrow image showed a marked hyperplasia of the erythroid, the ratio of granules and red was 1:1 or inverted to predominant red and the young and middle red blood cells predominant (the erythroid mature obstructed human nucleated red blood cells have giant red-like changes, granulocyte and megakaryocytes The system is suppressed.
4 nucleated red blood cell periodic acid-Schiff reaction (PAS reaction) strong positive; 5 except for various hemolytic anemia.
There is a large proliferation of young red blood cells in red blood disease, which should be distinguished from hemolytic anemia. In hemolytic anemia, the degree of erythrocyte proliferation is directly proportional to the degree of hemolysis. When red blood disease occurs, there are a large number of young red blood cells proliferating, and only mild hemolysis; when hemolytic anemia, the young red blood cells PAS reaction is weakly positive, while red blood disease is strongly positive; Radioactive radionuclide 5% iron utilization test, iron utilization rate is high in hemolytic anemia, and iron utilization rate is reduced in red blood disease.
When red blood disease, young red blood cells have giant red blood cell-like changes, which should be distinguished from megaloblastic anemia. Giant erythrocyte 3 blood, serum vitamin B. Reduce the level and use vitamin B. Or folic acid treatment responds well, and red blood disease is the opposite. The megaloblastic anemia is negative for PAS; nucleated red blood cells in the bone marrow are also significantly different in morphology.
There are two types of red blood disease:
1 acute red blood disease (Digu-glielmo disease) This type of clinical manifestations are similar to acute leukemia, the course of the disease has been severe, more than a few weeks to several months of death.
2 adult chronic red blood disease (Heilmeyer schonen type) clinical slow, the course of disease is about 2 years, and some cases can be subacute, the course of disease is 6 months.
According to the diagnosis of erythroleukemia, in addition to the above-mentioned diagnostic conditions of red blood disease, peripheral blood is often reduced in whole blood cells (sometimes white blood cells can also be significantly increased) and there are unequal numbers of immature granulocytes. Bone marrow shows erythroid hyperplasia with granulocyte hyperplasia, and granulocytes predominate with protoplasts and promyelocytes. Most of the course of the disease is acute and occasionally chronic. Erythemia should be distinguished from acute myeloid leukemia. The latter may also have young red blood cell hyperplasia, but the nucleated red blood cells in the bone marrow rarely exceed 1/4 of the nucleated cells, and the nucleated red blood cell PAS reaction is negative or weakly positive. Erythroleukemia should also be identified in the erythroleukemia-type leukemia response. The latter is mostly due to severe anemia. The extremely hyperplasia of the granules and red lines in the bone marrow is caused by extramedullary hematopoiesis, and the cause can be found. In China, 4 cases were reported as cases of spotted syndrome, which appeared 2 years after spleen spleen and 3 cases of thalassemia and primary liver cancer.
2, malignant lymphoma: in various types of malignant lymphoma, Hodgkin's disease granuloma type about 5% have splenomegaly, follicular lymphoma also often have splenomegaly. Reticulocytic sarcoma, Hodgkin's sarcoma and lymphosarcoma do not often have splenomegaly. Hodgkin's disease is characterized by a large number of cards, the so-called spleen-type Hodgkin's disease, the splenomegaly is extremely large and the whole body lymph nodes are not swollen. Spleen puncture helps to confirm the diagnosis. Bone marrow is sometimes available for evidence to confirm the disease.
3, malignant histiocytosis: this disease is often accompanied by varying degrees of splenomegaly, can be mild to extreme swelling. The pathological change of the disease is the proliferation of abnormal tissue cells in the hematopoietic tissue, which can be found in the bone marrow, spleen, lymph nodes, liver and perivascular tissue surrounding the blood vessels, and the lesion may involve one, more or all of the above-mentioned hematopoietic organs. Bone marrow smears found abnormal tissue cells, which is important for diagnosis, but one or more examinations did not find abnormal tissue cells, and the possibility of the disease could not be completely ruled out. If the patient has swollen lymph nodes, you can do lymph node biopsy to find abnormally proliferating tissue cells.
4, systemic tissue mast cell disease: is a systemic disease caused by abnormal proliferation of tissue mast cells. The cause is unknown and clinically rare. The main clinical manifestations were burnout, fatigue, fever, weight loss, rash, bleeding, anemia, bone pain and liver. Spleen, swollen lymph nodes, etc. Tissue mast cell infiltration can cause liver, card and lymphadenopathy and bone changes. The incidence of liver and splenomegaly is 40%-50%. Bone changes account for approximately 70%, including localized or diffuse osteosclerosis and osteoporosis.
Tissue mastocytosis can be divided into benign and malignant types, benign people can be divided into localized types, namely measles such as pigmentation (about 90%) and systemic tissue mast cell disease (only 10%) malignant type Mast cell leukemia is even rarer. The diagnosis of this disease is mainly based on the above clinical manifestations, as well as bone marrow smear or voice test to prove that there are a large number of tissue mast cells, and confirmed by histochemistry, or both X-ray bone signs.
5, idiopathic thrombocytopenic purpura: the disease splenomegaly is found in V5 cases, more manifested mild swelling, usually only 1-2 horizontal fingers under the costal margin. A bone marrow examination can confirm the diagnosis.
6, polycythemia vera: the disease can reach extreme splenomegaly. The disease has three characteristics: 1 face color brick red; 2 spleen enlargement; 3 red blood cell count and hemoglobin amount increased. Red blood cells (7.65-9.67) X 10/L, hemoglobin up to 200g/L. The platelet count was normal or increased, the clotting time was normal, and the bleeding time was normal or slightly prolonged. The disease must be differentiated from secondary polycythemia, secondary to frequent abnormalities due to hypoxia or erythropoietin, and many causes can be investigated.
7, hemolytic anemia: acute hemolytic anemia, the spleen often mild swelling; chronic hemolytic anemia, splenomegaly is often obvious, sometimes may be one of the main signs, splenomegaly is mild or moderate, Harder, no tenderness. The splenomegaly of thalassemia is extremely high in children. Adult cases are mostly mild and generally have mild splenomegaly. A series of laboratory tests for hemolytic anemia provide a basis for diagnosis.
8, bone marrow fibrosis: splenomegaly is extreme. Bone marrow puncture bone is particularly hard, and it is difficult to obtain bone marrow components, suggesting the possibility of diagnosis of this disease. A bone marrow biopsy can be finally diagnosed.
9, hypersplenism: is a syndrome. The main manifestations are: 1 one or more kinds of blood cells in the surrounding blood are reduced; 2 bone marrow hematopoietic cells are normal or hyperplastic, and are "mature blocked"; 3 splenomegaly may reach extreme; 4 blood card and bone marrow recovery after card cutting: normal. There are two types of primary and secondary spleen hyperfunction, rare in primary, such as primary splenomegaly, may be congenital diseases, and some cases have periodic cytopenia. Secondary infections can result from chronic infections, blood diseases, cirrhosis, connective tissue diseases, lipidoids, and the like.
(4) Connective tissue disease:
Systemic lupus erythematosus, dermatomyositis, nodular polyarteritis, Still's disease, and Eelty's syndrome may have mild splenomegaly. The incidence of systemic lupus erythematosus splenomegaly is 8.l%-17%, dermatomyositis is about 5%, and some nodular arteritis has splenomegaly. The cause of swelling is related to spleen embolism or arteritis in addition to cell proliferation. Still has liver, spleen and lymph nodes; Eelty is an adult rheumatoid arthritis with splenomegaly, anemia and neutropenia.
(5) Histiocytosis:
Including a group of lipid-like metabolic disorders, more common in 4 children, adults are rare. This group of diseases includes 5 syndromes, two of which are familial (high snow disease, Niemann-Pick disease) three are non-familial (Lei Xue Xue, Han Yi Xue Yi Ke syndrome, bone eosinophilic granuloma.
1. Le Yi Xue disease, Han Yi Xue Yi Da Syndrome (Yellow Lipoma) and bone eosinophilic granuloma have the same pathological changes, with abnormal tissue cell proliferation, eosinophils and foam cells. Infiltration, only in different numbers, can be considered as different manifestations of the same disease, so some of these three diseases are collectively referred to as "histiocylosis X" (histiocylosisX)
Clinically, patients have liver, splenomegaly and skeletal lesions suggesting the possibility of histiocytosis. Bone marrow biopsy or bone biopsy is helpful for diagnosis.
2, sea blue histiocytosis: a large number of sea blue tissue cells appear in the bone marrow. The typical shape is similar to that of Niemann-Pick cells, but there are a number of blue or green particles in the cytoplasm. The particles fill the entire cytoplasm for a long time and are opaque sea blue, hence the name. More common in women, clinically rare. Dimensionalization. The disease has both primary and secondary. The primary sexually transmitted disease is slow and the disease progresses relatively benign. Patients with multiple splenomegaly are unknown. Secondary can be secondary to primary thrombocytopenia and seek medical attention. Often accompanied by hepatomegaly, superficial lymph nodes are generally small. Purple epilepsy, chronic myeloid leukemia, polycythemia vera. Thrombocytopenia. Some cases have jaundice, abnormal liver function and hardening. The main reason for the diagnosis is the large amount of cirrhosis found in the bone marrow. There may be peripheral neuritis, pulmonary infiltration, and myeloid blue blood cells.
(6) Spleen tumor and spleen cyst
1, spleen malignant tumors: primary spleen malignant tumors are very rare. Pseudocysts are hemorrhagic, serum or inflammatory. It is also very rare to transfer malignant tumors to the card. Patients with primary malignant spleen pseudosplenic cysts have splenomegaly. The tumors of the tumor and spleen metastasis after mild trauma are characterized by large splenomegaly, hard surface and rapid increase of surface spleen. Large, physical examination in the left upper abdomen can reach the cystic mass, the unevenness has a tendency to progressively increase. The primary spleen metastasis cancer is soft, smooth, fluctuating, and has no mobility.9%-23%
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