malignant histiocytosis
Introduction
Introduction to malignant histiocytosis Malignant histiocytosis (malignant group) is a malignant proliferative disease of tissue cells in the mononuclear-macrophage system. The clinical manifestations are characterized by fever, hepatosplenomegaly, enlargement of whole blood cells and progressive failure. The evil group is more common in young adults, mostly in the 20 to 40 years old, and the incidence of men and women is 2~3:1. The disease can be divided into acute and chronic according to the course of the disease. Domestically, the acute type is more common, the onset is rapid, the condition is dangerous and the course of disease is short. Most cases without chemotherapy have died of liver and kidney failure, gastrointestinal tract and intracranial hemorrhage within 6 months of onset. . Due to the lack of specificity of symptoms, signs and laboratory tests, the current domestic diagnosis of this disease still relies mainly on clinical manifestations, bone marrow cell morphology and/or biopsy pathology. Early diagnosis is more difficult. basic knowledge The proportion of illness: 0.003% Susceptible people: more common in young adults, mostly in the 20 to 40 years old, male and female incidence is 2~3:1 Mode of infection: non-infectious Complications: anemia, gastrointestinal bleeding
Cause
Cause of malignant histiocytosis
(1) Causes of the disease
It is still unclear that it is generally considered to be a variant of histiocytic lymphoma or acute monocytic leukemia, which may be related to EB virus infection, and some people believe that it is an autoimmune proliferative disease or due to a deficiency in immune function. In recent years, it has been reported that the malignant group is often the second malignant tumor secondary to other tumors, often associated with malignant lymphoma (B cell), T cell and naked cell acute lymphocytic leukemia, acute granule-mononuclear Cellular leukemia, Lennerts lymphoma, is presumed to be involved in chemotherapy or primary tumor suppression of the immune system, leading to chromosomal abnormalities, clonal malignant mutations, and the occurrence of this disease may be related to the patient's immune function, due to mast cell proliferation in some cases during autopsy Some people think that it is an autoimmune proliferative lesion, which is initially allergic and gradually transforms into a tumor. Some people have noticed that the serum Epstein-Barr virus antibody titer is increased in patients with malignant group. It is suspected that this disease is related to viral infection. It is known that Epstein-Barr virus is caused by Burkitt. The cause of lymphoma, but the role played in the cause of the evil group is still unclear, some people have noticed the recent evil The incidence of the group in Central Africa has increased, presumably related to environmental factors, especially with viral factors, but there is still no strong serological and epidemiological evidence, Kobari et al observed a case of chronic EBV infection after the occurrence of evil In the group, the EBV was localized to the lymphocyte membrane antigen, and the EBV in the DNA was detected by in situ hybridization (ISH). The results showed that the EBV particles and the subsequent evil in the non-malignant tissue cells in the early chronic infection were found. The EBV particles in the cells are the same, and it is inferred that the normal normal tissue cells may become abnormal cells after EBV infection, and clonal expansion occurs, resulting in a vicious group. Domestic Liang Ping (1984) on 8 cases of malignant bone marrow specimens Electron microscopic observation showed that type IV nucleosome was found in 2 cases of malignant cells, which was considered to be a morphological sign that the cells had been affected by the virus. Some people in foreign countries suspected that the disease was related to genetic factors, and there were reports of the incidence of father and son. Domestic Guan Min et al. (1990) reported that 2 cases were siblings, and another brother in their family was sick. Three brothers were born in infants and young children. The condition was the same and both were within 3 months after the onset of illness. Death, evil group in recent years, domestic scholars have found in the lymphatic tissue autopsy showing severe atrophy, suggesting that patients with immune deficiencies, as immune deficiency is the cause or the result is inconclusive, in short, the cause is still not clear.
(two) pathogenesis
It is unknown at present. In 1969, Rodman reported that two cases had chromosomal translocations, namely t(2;5)(p23;q35). In addition, there was a chromosome 1 abnormality. Morgan et al. found a virulent cell line in 1986. The cleavage of the long arm of a chromosome (5q35 rupture), often accompanied by t(2;5), and another reported that chromosome 1, 3 and 6 also have translocations, Morris (1994) and other confirmed t (2) ; 5) can generate NPM / ALK fusion gene, Shiota et al (1995) pointed out that this fusion gene encodes a (80kD) high phosphorylated protein (p80), NPM / ALK fusion gene and p80 found in all t (2; 5) In the case, but the expression of p80 NPM/ALK is an independent disease. The determination of 5q35 rupture can be used as a premise for the diagnosis of CD30-positive tumors by cytogenetics or p80 expression. Wu Shiqi et al. (1983) proposed group D. One chromosome loss is the most common karyotype of this disease. In 1989, Stephen et al reported three cases of bad group, all of which expressed ki-1 (CD30), all of which had t(2;5)(p23;q35) translocation. It is believed that this translocation may be a common feature of a group of diseases characterized by evil groups. In 1990, Abe et al studied the cytogenetics of four evil groups, two of which had chromosome 17 abnormalities. Pointed on the short arm 13 (17p13), reviewing the literature, another 7 cases of the same group of abnormal findings, it has been proved that the p53 gene mutation is higher in human malignant tumors, and p53 (tumor suppressor gene) is located in On the short arm of chromosome 17, in 1992, Tian Hong et al. performed bone marrow chromosome analysis on two patients with malignant leukemia. All of them had partial trisomy 1 (1qter-1p11) and 1p11 break translocation.
Prevention
Malignant histiocytosis prevention
1. Assist patients in their daily lives, reduce oxygen consumption, and reduce heart and lung burden.
2. Ensure adequate rest and sleep time for the patient, keep the environment quiet and comfortable, avoid unnecessary operations, reduce interference factors such as noise, visitors, keep warm, and avoid cold.
3. Always discuss with the patient methods that can prevent or reduce fatigue, such as avoiding predisposing factors, keeping the condition stable, cooling, pain relief, timely replacement of sweaty clothes, and being served.
Complication
Malignant histiocytosis complications Complications, anemia, gastrointestinal bleeding
The complications of this disease are high heat exhaustion, hemorrhage and infection. In severe cases, death can occur. The disease group often involves multiple organs, especially acute type. The onset is urgent, the course is short and dangerous, and fever, anemia, and bleeding may occur. , liver, spleen, swollen lymph nodes, cough, chest pain, dyspnea, abdominal pain, gastrointestinal bleeding, etc. These are its clinical manifestations, and it is also a complication, it is difficult to distinguish in clinical.
Symptom
Symptoms of malignant histiocytosis common symptoms, bloody aging, accelerated irregular hot nasal ecchymosis, relaxation, hot gum, bleeding, intestinal perforation
According to the different infiltration sites of the evil group, there are different clinical manifestations. From the above pathological involvement, hematopoietic tissue can be involved (most common), and non-hematopoietic tissue can be involved. Therefore, the clinical manifestations are diverse. Many classification opinions are proposed. For example, according to Israel, there are 5 types. In fact, most of the pathological groups have pathological features, and the clinical manifestations have characteristics of type 2 or above. Although Cazal reduces it to visceral, skin type, and child type, it is considered that children and adults. The clinical difference is not large. It is suggested that the lesion mainly involves hematopoietic tissue, which is called common type. The main type of non-hematopoietic tissue is called special type. There are more special types, such as skin type, gastrointestinal type, lung type, kidney type and nerve. Type, multiple serositis type, intestinal perforation type, spleen and spleen spontaneous rupture type, chronic recurrence type, etc., some authors believe that clinical manifestations are diverse, difficult to summarize, clinical and pathological control is not consistent, think The classification has little significance for the prognosis. There is no unified opinion on the treatment of domestic classification. In addition, it is proposed to be acute and chronic according to the course of the disease (more than 1 year is chronic), because chronic is very Rarely, most cases have an acute onset and a short course of disease. Therefore, there is no clinical emphasis on urgency or slowness. In 1975, at the symposium held in Sanming City, Fujian Province, the chronic type was not conclusive, and the domestic literature reports (including special A comprehensive analysis of 862 cases of the type is as follows to understand the diversity of clinical manifestations.
First symptoms
Regardless of the type of evil group, fever is often the first performance, the heat type is mostly irregular high heat (38.7%), followed by the heat of retention (26.3%), relaxation heat (21.2%), intermittent heat (10.8%) And low fever (3%) is rare, pale, fatigue is caused by anemia, bleeding tendency is not uncommon in the first performance, especially in the late stage of the disease is one of the causes of death, jaundice is generally not obvious in the early days Later, some patients were admitted to infectious diseases hospitals with the first jaundice. In addition, many cases of fever, abdominal pain and repeated bloody stools were reported in China, which were often accompanied by intestinal perforation and were diagnosed as acute abdomen. There are reports of cases of pneumonia, lung cancer, and tuberculosis in the lungs, and there are many reports of paraplegia, cranial nerve palsy or increased intracranial pressure. It is worth mentioning. Some patients have bones and joint pains are often very significant, and painkillers are often ineffective.
The disease can be divided into acute and chronic according to the course of the disease.
(1) fever is the most prominent performance, more than 90% of patients with fever as the first symptom, body temperature can be as high as 40 ° C or more, the heat type is more irregular heat, there are intermittent heat, relaxation heat and retention heat, a few cases Antibiotics can temporarily reduce body temperature, but more cases of fever are related to the disease itself, and do not respond to antibiotic treatment. Although corticosteroids have a cooling effect, they are not persistent, and only when the chemotherapy is effective, the body temperature can return to normal.
(2) Anemia is also one of the more common symptoms. Anemia occurs in the early stage of acute type, which is progressively aggravated. In advanced cases, paleness and systemic failure are very significant. In a few cases with slow onset, the earliest prominent symptoms may be anemia. And fatigue.
(3) bleeding is more common with skin spots or ecchymoses, followed by nasal discharge, bleeding gums, mucous membranes, blood in the urine, hematemesis or blood in the stool.
(4) In addition, fatigue, loss of appetite, weight loss, and weakness are also significant as the disease progresses.
2. Signs
Large liver, splenomegaly is a common sign, often mild or moderate swelling, 1 case of the liver is 10 cm below the right rib; 1 case of the spleen is the largest pelvic cavity, but in the gastrointestinal type of the disease about 1/3 of the liver The spleen is not touched, 2/3 swollen is mildly swollen, superficial lymph nodes are generally like large soybeans, peanuts are large, and the maximum is as large as eggs. From the autopsy, the evil group mainly invades the deep lymph nodes, and the superficial lymph nodes are not. Large and can not rule out the evil group, the skin damage is most common with nodules and masses (can form granuloma or cause fibrosis, form nodules on the naked eye, but the cells under the microscope do not stick together), and may be accompanied by ulcers, still May be associated with non-specific damage, such as maculopapular rash, purpura and erythroderma, skin damage is more common in children with evil, and is considered to be characteristic, the lungs may have a voice with cough, shortness of breath, abdominal touch Most of the lumps were signs of intestinal type evil group. Some people counted 53 cases with bowel symptoms (except fever, abdominal pain, diarrhea), 41.5% of abdominal masses, and 52.8% of intestinal perforations.
Examine
Examination of malignant histiocytosis
Laboratory inspection
Peripheral blood
At the time of the patient's visit, only 10% of the blood is normal, and the rest have various degrees of blood cell reduction (the white blood cell count can be increased in a few cases). With the development of the disease, the whole blood cell reduction becomes more and more obvious, which is one of the prominent manifestations of this disease. About half of the patients are likely to find abnormal cells at the end of the peripheral blood (especially in concentrated or in the white blood cell layer).
2. Bone marrow
In the bone marrow smear, most of the normal hematopoietic tissues were still observed. 241 cases of bone marrow smear were analyzed, 66.4% of the bone marrow was active or significantly active; 33.6% showed hyperplasia or severely reduced, because the lesion was unevenly distributed. Sometimes, many bone marrow punctures fail to find malignant cells. When various malignant cells are mixed in the bone marrow smear, they may vary in size and pleomorphism. The changes in cytological morphology are not the same at home and abroad. At the National Blood Academic Conference, according to the characteristics of cell morphology, they were divided into the following types:
(1) Abnormal "tissue" cells (malignant "tissue" cells): large (20 ~ 40m) shape regular or irregularly round, cytoplasm is more abundant than normal primordial cells, cytoplasm dark blue or light blue, dark blue There are often no particles, light blue can have a few or more fine particles, there can be a number of vacuoles, the core can be round or oval, sometimes branched, occasionally dual-nuclear, fine nuclear chromatin Or in the form of a network, the nucleolus is different.
(2) Lymphoid "tissue" cells: such as lymphocyte size, shape similar to lymphocytes or endothelial cells, can be elliptical, irregularly round or narrow with a curved tail, cytoplasm is light blue, gray blue Color, containing more fine particles, the core is often biased to one side or one end, the nuclear chromatin is more detailed, occasionally nucleoli.
(3) Single-nuclear "tissue" cells: the morphology resembles monocytes, but the nuclear staining is deeper or coarser.
(4) Multinucleated giant cells: very large, diameter up to 50m, irregular shape, cytoplasmic light blue without particles or a few small particles, usually containing 3 to 6 nuclear or nuclear components, nucleolus or hidden or Obvious.
(5) phagocytic cells: large volume, mononuclear or dinuclear, elliptical bias, loose chromatin, clear nucleolus, cytoplasm containing phagocytic mature red blood cells or fragments thereof, young red blood cells, platelets and neutrophils Cells, etc., a phagocytic cell can devour more than 20 blood cells.
According to our observation, abnormal "tissue" cells and/or multinuclear "tissue" cells have specific diagnostic value, while lymphoid and mononuclear cells can also occur in other diseases, without specific diagnostic significance, as for bone marrow coating. There are many abnormal "tissue" cells in the film that can be diagnosed. There is no clear regulation. In 1959, Yu Zhifei reported 18 cases, and the abnormal tissue of the bone marrow accounted for 10.5% of the nuclear cells, up to 88%.
3. Histochemical staining
The cytochemical reaction of the special cells of this disease is mostly negative for peroxidase staining, negative or weakly positive for Sudan black staining, and weakly positive diffusion reaction for glycogen staining; acid phosphatase staining is mostly moderately strong positive reaction can be tartaric acid Inhibition, glucuronidase staining weakly positive ~ moderately positive reaction; non-specific esterase staining positive ~ strong positive reaction, can be inhibited by sodium fluoride; -ASD chloroacetate naphthyl esterase and alkaline phosphatase negative reaction, lysing Enzymatic staining was positive, 1-antitrypsin and 1-antitrypsin were positive.
4. Biochemical examination
62% serum alanine aminotransferase increased; 54.3% urea nitrogen increased; in some cases lactate dehydrogenase, alkaline phosphatase increased, serum ferritin content increased significantly.
5.47.6% increased erythrocyte sedimentation rate and decreased neutrophil alkaline phosphatase.
Auxiliary inspection
Pathological examination
Lymph node skin, liver, bone marrow and other pathological biopsy found the evil group cells.
2.X line
Chest X-rays have changed, such as: diffuse or interstitial infiltration, scattered miliary and small nodular shadows, or mediastinal hilar lymph nodes, pleural effusion, etc., but X-ray performance is generally non-special The opposite sex, often with complications.
3. CT, MRI examination
Found skull, abdomen, liver, spleen, retroperitoneal lymph nodes and chest and other lesions.
4.B Ultra
Hepatic spleen lymph node enlargement, pleural effusion, ascites and so on.
Diagnosis
Diagnosis and differentiation of malignant histiocytosis
diagnosis
In the past, the diagnosis of this disease mainly relied on clinical manifestations and cell morphology or biopsy findings. In recent years, there has been a new understanding of the malignant cell source of this disease, and even proposed that the disease name should be changed, but as the same disease entity, its Clinical manifestations and cell, pathological findings should not differ from past descriptions, so the past diagnostic basis is still used, just need to add some cell markers, cellular and molecular genetic indicators to determine the source of malignant cells, in order to be accurate Name the disease.
Clinical manifestation
Acute onset, long-term fever, progressive hepatic and spleen lymphadenopathy, progressive liver and kidney failure, progressive whole blood cell reduction, progressive systemic failure, often accompanied by jaundice, hemorrhage, skin damage and serosal cavity Fluid.
2. Bone marrow smear
A certain number of abnormal cells mentioned above must be found in the diagnosis. Because the bone marrow is not always involved, and the lesions are often focally distributed, the bone marrow may not be excluded from the test 1 or 2 times. It is necessary to repeat the examination several times. The positive rate was higher than that of the tibia (4 cases of tibia negative were changed to sternal puncture, and several cases of sacral smear abnormal cells accounted for only 1%, and for sternal puncture, abnormal cells accounted for 8% of nucleated cells).
3. Peripheral blood concentrated smear examination
Between 305 cases of peripheral blood smears, 152 cases (49.8%) found abnormal cells, and the chance of finding abnormal cells after blood concentration was higher. The blood concentrated smear could complement the bone marrow smear.
At present, the main method for diagnosing the evil group is to rely on bone marrow puncture smear examination. The success rate of bone marrow diagnosis is 69%. The bone marrow finds "bad group" cells occupy 2% to 78% of nuclear cells (of which 2% to 20%) 77.1%, accounting for 72% or more, 22.9%). It is suggested that through 5 bone marrow punctures, 98% of cases can be diagnosed. In fact, 10.2% of patients are still undiagnosed before birth, and another 10.3% of patients need to rely on Pathological examination of other parts of the living tissue (Table 4), the success rate of confirmed diagnosis of other parts of the living tissue: skin biopsy 72.7% (24/33); lymph node biopsy 63.1% (70/111); bone marrow biopsy 39.7% (27 /68); liver biopsy 25% (5/25), in addition to individual lymph node puncture, splenic puncture, pericardial puncture, lung puncture, cerebrospinal fluid and chest and ascites to find "malignant" cells, superficial lymph node biopsy success rate is lower than the skin Biopsy is due to the dignity group infringing deep lymph nodes, and bone marrow biopsy and liver biopsy can only be used as auxiliary diagnosis because the malignant group often presents focal infiltration, and the puncture is difficult to grasp.
In short, the diagnosis must emphasize the importance of clinical manifestations. Many domestic scholars agree with the author (1973) that clinical diagnosis of this disease and morphological support can be initially diagnosed; clinical suspicion without morphological changes cannot rule out the disease Multiple site bone marrow puncture (positive rate of sternal puncture may be higher) and possible biopsy should be repeated; those with morphological features but not consistent with clinical manifestations should be differentiated from reactive and other histiocytosis diseases. To determine the source of malignant cells, a variety of cytochemistry, immunological markers, and cellular and molecular genetic tests should be performed, Ki-30 (CD30) positive, t(2:5) (p23:q35) chromosomal translocation, fusion gene NPM /ALK (p80), help to identify anaplastic large cell lymphoma, other markers such as epithelial membrane antigen (EMA) and CD25 (IL-2 receptor) may also be positive.
Differential diagnosis
Malignant histiocytosis should be differentiated from diseases such as reactive histiocytosis, acute leukemia, bone marrow metastasis, and aplastic anemia.
The clinical manifestations of this disease are diverse and non-specific, so it is easy to misdiagnosis based on clinical manifestations. The comprehensive misdiagnosis rate of 669 cases reported by domestic comprehensive reports is as high as 69.4%, and there are more than 70 misdiagnosed cases reported in domestic literature. Misdiagnosed situation:
1 diagnosed as aplastic anemia, acute leukemia, etc. due to fever, bleeding, and complete blood cell reduction;
2 due to fever, jaundice, liver and spleen and misdiagnosed as jaundice hepatitis, cirrhosis, biliary tract infection, etc.;
3 due to fever continued to retreat with gastrointestinal symptoms and mistaken for typhoid, sepsis, intestinal tuberculosis, etc.;
4 considered as intestinal tumor, lymphoma, ulcerative colitis, etc. due to blood in the stool, diarrhea or abdominal mass;
5 due to fever, cough, shortness of breath, pleural fluid and mistaken lung infection, lung cancer, tuberculosis, etc.;
6 due to fever, hematuria, edema and diagnosis of glomerulonephritis, urinary tract infection, and even uremia;
7 due to fever with paraplegia, cranial nerve palsy or convulsions and mistaken for encephalitis, myelitis, intracranial tumors;
8 due to fever accompanied by pericardial effusion or pleural effusion and misdiagnosis as multiple serositis;
9 mistaken for necrotizing granuloma due to nasopharynx ulcer;
10 due to fever, skin nodular ulcers or erythema and mistaken skin tuberculosis, non-suppurative panniculitis, mycosis fungoides, etc. In addition, many cases have been surgically investigated for surgical diseases, so the disease should be improved Vigilant.
The support of cell morphology with bone marrow can help diagnose, but many non-neoplastic diseases can also cause reactive tissue cell enlargement, often identified with this disease.
1. The concept of reactive histiocytosis
It refers to the presence of obvious primary disease, due to the stimulation of certain factors of the primary disease, tissue cell proliferation occurs, after the primary disease is removed, the tissue cells proliferating after stimulation are alleviated naturally, when the primary disease diagnosis is clear When the degree of histocompatial hyperplasia is light, the latter is easily neglected. However, if the patient with severe disease has strong tissue cell response, the diagnosis of the primary disease is misdiagnosed as evil group. Some infectious diseases are known. Tuberculosis, typhoid fever, brucellosis, malaria, etc. can cause bone marrow tissue cells to increase, and phagocytosis of blood cells. In the past, the concept and classification of tissue cell proliferation was confusing. In the 1970s, reactive histiocytosis was divided into 3 class:
1 Infectivity: secondary to infectious diseases, including the above diseases and hepatitis, infectious monocytosis, etc.;
2 allergic reactivity: secondary to connective tissue disease, drug allergy, cutaneous inflammatory lymphadenopathy and certain vaccination reactions;
3 malignant neoplastic disease: there may be tissue cell proliferation when not transferred to the bone marrow.
In the early 1970s, Zhang Zhinan and other follow-up work found that some cases of the original diagnosis of the evil group survived after several years, and some developed typical rheumatoid arthritis; some later found to be lupus erythematosus; The findings are prolonged hepatitis; some have no signs of disease, review these cases, past clinical manifestations and bone marrow cell morphology, the results: clinical onset is very similar to the "malignant group", with high fever, pale, liver and spleen, all Blood cell reduction, individual bleeding tendency, 1 case of blood pressure has dropped to 80/57mmHg, automatic discharge, most of the disease is very dangerous, but the morphology of bone marrow cells are mostly mononuclear and lymphoid cells, some have atypical cells, but not many More (not more than 1%), only 1 case is more (about 20%), blood pressure is automatically discharged from the hospital, there is no joint symptoms at that time, the joint deformed and deformed many years later, it is rheumatoid arthritis, which can be seen Individual reactive tissue cell proliferation and "bad group" are difficult to identify at an early stage.
In 1979, Risdal et al reported 19 cases of tissue cell proliferation due to viral infection and phagocytosis of blood cells. Among them, 14 cases had been treated with long-term immunosuppressive agents before the onset of the disease, and the virus was positive, which was called virus-related hemophagocytic syndrome. Virus-associated hemophagocytic syndrome (VAHS), after which it was found that not only viruses can cause hemophagocytic syndrome, but also many bacteria, fungi, and even recent blood transfusions, tumor dissemination, etc. can also cause infection-associated hemophagocytic syndrome (infection- Associated hemophagocytic syndrome), known as hematophagic histiocytosis, in addition to hematophagic syndrome, is derived from the perspective of bone marrow cytology, manifested as tissue cell proliferation and active Engulf various blood cells.
Many pathogens have been identified to cause hemophagocytic syndrome:
1 virus, such as Epstein-Barr virus, cytomegalovirus, herpes simplex virus, varicella zoster virus, adenovirus, parvovirus B19;
2 bacteria, such as intestinal Gram-negative bacilli, Haemophilus influenzae, Streptococcus pneumoniae, Staphylococcus aureus, Brucella abortus, Mycoplasma pneumoniae, etc.;
3 Bernard Rickettsia;
4 Mycobacterium tuberculosis;
5 fungi, such as capsular histoplasma, Candida albicans, Cryptococcus neoformans.
6 Leishmania, the clinical manifestations of hemophagocytic syndrome vary with the primary disease, the most common common symptom is fever, mostly with high fever, can be accompanied by chills, night sweats, anorexia, weight loss and liver, Spleen, lymph nodes are slightly enlarged, some patients may have bleeding, rash, whole blood cell reduction, liver function damage or coagulopathy, VAHS may have central nervous system symptoms, lung infiltration or renal failure and other organ damage symptoms VAHS often has symptoms of viral infection 2 to 6 weeks before the disease, although the hemophagocytic syndrome is essentially a reactive histiocytosis. If there is no secondary infection, it will naturally relieve within 1-8 weeks, but it is serious. Cases, the clinical process is very similar to the "bad group", the mortality rate can be as high as 30% to 40%, the degree of danger is almost difficult to distinguish from the "bad group", such as Chen et al. in 1991 reported 10 cases of Taiwanese children caused by EB virus Explosive VAHS, clinical manifestations of fever, jaundice, hepatosplenomegaly, complete blood cell reduction, coagulopathy and liver dysfunction, bone marrow examination with atypical T lymphoid cells and a small number of B immunoblasts infiltration, There were mature tissue cell proliferation with phagocytic blood cells. The first 6 cases were diagnosed as malignant group, and 10 cases died rapidly. The average survival time from fever to death was 16 days, mainly due to coagulopathy with multiple organ failure and chance. Pathogen infection, confirmed by serological identification and blot hybridization experiments, was an acute infection with Epstein-Barr virus.
2. Laboratory examination of reactive histiocytosis
(1) Peripheral blood cells were reduced to varying degrees: 828.4 cases of reactive histiocytosis in the country had anemia of 48.4%; complete cytopenia accounted for 14.6%; the rest were thrombocytopenia or leukopenia or the third line of the three lines Reduced, a small number of white blood cells increased (7.3%); peripheral blood smears found mature tissue cells accounted for 17.8% (1% to 31%), foreign data anemia, white blood cells and thrombocytopenia were 9l%, 80% And 88%.
(2) Bone marrow smear shows increased tissue cells: most of the cells are mature or mononuclear and lymphoid tissue cells, which may be accompanied by blood cells being phagocytized. In some cases, a small number of abnormal tissue cells or 1 or 2 multinucleated giant cells can be seen. At present, it is considered that the whole blood cell reduction is not caused by the excessive phagocytosis of blood cells by the tissue cells, but the result of infection inhibiting the bone marrow.
(3) Other laboratory tests: often elevated serum alanine aminotransferase, may have azotemia, or increased blood bilirubin, prolonged prothrombin time, hypogammaglobulinemia, increased blood alpha 2 globulin ratio , lactate dehydrogenase increased, etc., VAHS patients serum serum antibody titer can be increased, virus culture can be positive, other reasons for reactive tissue cell proliferation, can appear sooner or later signs of the primary disease and the corresponding experimental findings.
3. Identification of malignant group and reactive histiocytosis
In 1994, Feng Yun et al reported the similarities and differences between 13 cases of hemophagocytic syndrome and "malignant group" disease diagnosed between 1986 and 1992.
It can be seen that there are sometimes difficulties in distinguishing between clinical manifestations and cell morphology, and pathogen examination and serum ferritin detection may be helpful in identifying the two. It is considered that serum ferritin is significantly higher in the "bad group" than in reactive tissue cells. Hyperplasia, but it has also been reported that the serum ferritin of both diseases is significantly increased, there is no significant difference between the two, in addition, the positive rate and integral of neutrophil alkaline phosphatase (NAP) in the "bad group" is extremely low However, most of the reactive NAP is increased. It is believed that the cells of the "malignant group" have poor mitotic figures, and the phenomenon of phagocytic blood cells is less common, while the reactive tissue cells are more mature, with few mitotic figures, and phagocytic cells. The phenomenon is extremely obvious. Our past experience shows that in the absence of obvious infection, the adrenal cortex hormone can be tested. The reactive person can take the medicine for 2 to 3 days and the body temperature can be gradually regressed and can be stopped in a short period of time, although there are a few patients. Re-heating, but there may be a longer interval, and the body temperature can still be retreated. It depends on whether the primary disease has been treated at the same time. We have performed 38 cases of reactive histiocytosis. Analysis, 16 cases of primary disease can not be determined, 9 cases of natural temperature without natural treatment, 7 cases of oral prednisone after heat retreat, liver spleen and lymph nodes shrink after heat retreat, blood and bone marrow gradually return to normal, and evil The group responded poorly to adrenocortical hormone. Even if the body temperature decreased, it often failed to fall to normal or decreased in a short period of time, and the continuous application gradually failed. It has been reported that some hemophagocytic syndrome is effective in the application of cyclosporin A. It is emphasized that the increase of tissue cells in the bone marrow with phagocytosis should first exclude the responsive ones. Because more common, more than half of the responders are more likely to find the primary disease, and the general reactive tissue cell growth is not long. Repeated bone marrow puncture examinations have changed a lot and disappeared quickly. Once the abnormal tissue cells in the bone marrow appear in the evil group, the abnormal cells of repeated bone marrow puncture will gradually increase.
