Pediatric Hodgkin's Disease
Introduction
Introduction to Children's Hodgkin's Disease Hodgkins disease is a malignant tumor of the lymphatic network that often occurs in a group of lymph nodes and spreads to other lymph nodes and/or extranodal organs or tissues. The clinical manifestations of this disease are fever, fatigue, anorexia, mild weight loss, itching, and unexplained fever. basic knowledge The proportion of the disease: the incidence of this disease in a specific population is around 0.1% Susceptible people: children Mode of infection: non-infectious Complications: thrombocytopenia
Cause
The cause of Hodgkin's disease in children
The etiology and pathogenesis of HD are unknown, and many studies suggest that viral infections and immune abnormalities may be the causative factors.
Infection factor (25%):
Many animals such as chicken, rat, mouse, cat and bovine lymphoma can be caused by viruses, usually RNA viruses. Currently, Burkitt's lymphoma is proven from epidemiology, virology and immunology. Closely related to Epstein-Barr (EB) virus, the serum EB virus antibody titer is significantly increased in most children with Burkitt's lymphoma, and HD is related to the past history of infectious mononucleosis and EB virus exposure. The risk of HD in patients with infectious mononucleosis caused by Epstein-Barr virus is three to four times higher than that of the general population.
Immunity factor (25%):
Some congenital immunodeficiency diseases, such as non-gammaglobulinemia, are more likely to develop lymphoma; those who receive immunotherapy in kidney transplantation have a 200-fold higher risk of developing tumors than normal people, and one-third of them are lymphomas. Such as rheumatoid arthritis, systemic lupus erythematosus, immune hemolysis and other autoimmune diseases, easy to develop lymphoma, HD patients often have lymphocyte conversion rate and rosette formation rate decreased, skin delayed hypersensitivity negative cells Immunodeficiency showed a decrease in IgG, IgA and IgM during the progression of the disease, and a significant decrease in IgM, especially in the case of lymphopenia, indicating that humoral immunity is also defective.
Genetic and environmental factors (30%):
It has been reported in the literature that HD and other malignant lymphomas can occur in multiple members of a family. It is estimated that the risk of developing a disease in a close relative of a HD patient is 3-5 times higher than that in the general population. The risk of HD in monozygotic twins The sex is 100 times higher than that of the general population, but there is no increased risk in heterozygous twins. It has been reported that histocompatibility antigen (HLA-A) is significantly associated with HD, namely HLA-A11 and W5, HLA- B7 and W15 are susceptible to HD, and some diseases with chromosomal abnormalities, such as congenital type, congenital testicular hypoplasia, the incidence of lymphoma is high, some data show that the occurrence of HD may be related to the environment; also reported It occurs in association with taking phenytoin (Dalendin) and contacting livestock, especially rabbits.
Pathogenesis
There are often normal lymphocytes, plasma cells, eosinophils, tissue cell reactive infiltration, RS cells with abnormal cell morphology, RS cells are large and deformed, diameter 1545m, rich in cytoplasm, multinucleated Or multi-leaf nucleus, the nuclear membrane is deeply stained, and there is a fine chromatin network, which forms a light-stained circle around the nucleolus. The nucleolus is large and obvious. It is difficult to diagnose the disease when no RS cells are seen, but in others. Similar cells can be seen in diseases such as infectious mononucleosis, non-Hodgkin's lymphoma and other non-lymphoid malignancies.
According to the RYE classification system, HD is divided into 4 histological subtypes:
1. Lymphocyte predominant (LP): 10% to 15%, more common in boys and younger children, clinical lesions are often more limited.
2. Mixed cellularity (MC): more common in children under 10 years old, RS cells are easier to see, the above various types of reactive cell infiltration, visible focal necrosis and fibrosis, clinical lesions often range Widely associated with extranodal lesions.
3. Lymphocyte depleted (LD): rare in children, more common in HIV-infected patients, a large number of abnormal malignant reticular cells and RS cells in the lesion, lymphocytes are rare, extensive necrosis And fiber stoves.
4. Nodular sclerosing (NS): The most common in children, easy to see RS cells, lymph nodes with envelope, collagen band extending from the capsule to divide the lymph nodes into multiple nodules, clinically the following neck On the collarbone, the mediastinum is more common.
Prevention
Pediatric Hodgkin's disease prevention
1. Avoid contact with harmful factors: Avoid exposure to harmful chemicals, ionizing radiation and other factors that cause leukemia. When exposed to poisons or radioactive materials, strengthen various protective measures; avoid environmental pollution, especially indoor environmental pollution; pay attention to rational use of drugs, caution Use cytotoxic drugs, etc.
2. Vigorously carry out prevention and treatment of various infectious diseases, especially viral infectious diseases, and do a good job of vaccination.
3. Do a good job in eugenics, prevent certain congenital diseases, such as 21-three-body, Fanconi anemia, etc., strengthen physical exercise, pay attention to food hygiene, maintain a comfortable mood, work and rest, and enhance the body's resistance.
Complication
Complications of Hodgkin's disease in children Complications thrombocytopenia
Mediastinal mass, trachea, bronchial compression symptoms, anemia, etc., with thrombocytopenia, thrombocytopenia and bleeding tendency.
Symptom
Symptoms of Hodgkin's disease in children Common symptoms Cervical lymphadenopathy thrombocytopenia Brushing bleeding Superficial lymph nodes progressive enlargement
1. Age of onset and gender
School age and preschool children have more morbidity. Most of the reported minimum ages are 2, 3 years old, mostly children over 2 years old. Occasionally, infant cases are reported. Males are significantly more than females, and the ratio of male to female is more than 3:1.
2. Pathogenesis and primary damage
The disease is often caused by lymph nodes, characterized by painless lymphadenopathy, but it has a "rubbery feeling" when touched. The swollen lymph nodes often exist for weeks or months, and the increase or decrease is not related to whether or not antibiotic treatment is given. Most children with onset of asymptomatic cervical lymphadenopathy, accounting for 60% to 90%, and spread along the adjacent lymphatic pathway, the progress of the disease is slow; sometimes the lesion can jump from above the horizontal to the horizontal, and first Involved in the spleen and splenic lymph nodes; a few started with intractable cough, X-ray examination found mediastinal mass, also due to relaxation of hyperthermia or multiple groups of shallow lymph nodes slightly enlarged, confirmed by biopsy, occasional primary damage In deep lymphoid tissues, sometimes with fever, anemia and other systemic symptoms are already obvious, but it is still difficult to determine the diagnosis.
3. Systemic symptoms
Non-specific symptoms include fever, fatigue, anorexia, mild weight loss, itching, unexplained fever above 30 °C or periodic fever, weight loss of more than 10% within 6 months, a large number of night sweats should think of this disease.
4. Lymph node enlargement
Painless clavicle, lymph node enlargement in the neck or other parts is the most common, lymph node hard and rubbery feeling, about 2 / 3 of patients have different degrees of mediastinal infiltration at the time of treatment, causing cough and other trachea, bronchial compression symptoms The tumor originates from the clavicle, and the neck is more common. The underarm, inguinal, and abdominal lymph nodes are relatively rare in the primary. When the mass is enlarged, the compression of the relevant part may occur.
5. Can be combined with immune dysfunction
Such as combined with immunological hemolytic anemia, anemia, jaundice, reticulocyte elevation, Coombs test positive, with immune thrombocytopenia, thrombocytopenia, bleeding tendency, increased platelet-related antibodies, bone marrow megakaryocyte maturation disorders.
6. Clinical staging
Accurate clinical staging is the premise of formulating the correct treatment plan and estimating the prognosis. Staging can be divided into clinical staging, which refers to the scope of invasion found in clinical examination; pathological staging (PS), including laparotomy, surgical sampling and bone marrow biopsy The scope of the violation found.
(1) International staging: At present, the staging plan modified by the Ann Arbor meeting in 1971 is adopted at home and abroad. The meeting was supplemented and revised at the meeting held in Cotswalds, England in 1989. It includes features that were not found in the original Ann Arbor staging. , such as the form of expression, age, gender, evaluation of mediastinal mass, serum LDH, albumin, total lymphocyte count and number of invading lymph nodes, in addition to age, the international staging correction program is also applicable to children, according to the anatomy of the lesion invasion The purpose of defining the broad extent of disease distribution for staging is to provide a basis for clinical treatment.
(2) Clinical stage (CS): Each stage is also divided into two groups according to the presence or absence of systemic symptoms. Those with no systemic symptoms are group A, and those with group system are group B.
1 systemic symptoms include:
A. Weight loss, no more than 10% weight loss for other reasons within 6 months before the visit.
B. Fever, unexplained fever, body temperature is often above 38 °C.
C. Night sweats, sweating at night or falling asleep.
2 Inspection items and staging procedures: The inspection items and staging procedures required for clinical staging are as follows:
A. Routine inspection items:
a. Medical history: pay special attention to the presence or absence of "B" group symptoms, examination and review of living tissue specimens.
b. Total physical examination: pay special attention to lymphadenopathy, including lymph nodes on the trochlear, pharyngeal lymphatic ring, hepatosplenomegaly, and tenderness of the bone.
c. Laboratory tests: blood cell count, erythrocyte sedimentation rate, urea nitrogen, uric acid, electrolytes, liver function determination, bone marrow aspiration and biopsy.
d. Radiological examination: chest photography, bilateral lymphangiography, venography.
B. Check items if necessary: CT scan of the chest and abdomen; exploratory laparotomy, lymph node and liver biopsy, spleen resection; bone radiography, radionuclide bone imaging; 67Ga scintigraphy.
Cational emission tomography (PET): Evaluation of HD is an important tool, but it is not routinely used like magnetic resonance imaging (MRI). For a disease with a clear site, the positive rate is 86%. It is also found that diseases that cannot be detected before and can be diagnosed by biopsy have an important effect on the monitoring of minimal residual lesions, extranodal lesions, metastasis and recurrence after treatment.
(3) Pathological stage (PS): refers to the range of lesions found in addition to the above-mentioned clinical examinations, plus the following items.
1 bilateral bilateral iliac spine bone marrow biopsy.
2 laparotomy, including splenectomy biopsy, liver puncture and wedge biopsy, as well as abdominal aortic, mesenteric, hilar and splenic lymph node biopsy, if necessary, laparoscopy can also be used instead of exploratory laparotomy.
It should be noted that not all patients require pathological staging, only when developing a treatment plan, need to know whether there are hidden intra-abdominal lesions and spleen invasion, only need to do laparotomy; if the proposed treatment is not determined by the details of abdominal lesions, then it is not necessary Laparotomy is performed. In addition, with the promotion and application of PET technology, the necessity of exploratory laparotomy will be significantly reduced.
Examine
Pediatric Hodgkin's disease check
1. Hematological examination: There is no specific abnormality in blood routine examination. Occasionally, eosinophils or monocytes may increase, and erythrocyte sedimentation rate may increase.
2. Lymph node biopsy: Pathological histomorphometry is a necessary means of diagnosis.
3. Bone marrow biopsy: HD can cause focal bone marrow metastasis, so bone marrow biopsy is easier to find tumor cells than bone marrow smears, and bone marrow biopsy should be routinely performed before treatment.
Imaging examination, optional chest X-ray film, abdominal B-ultrasound, chest CT, abdominal CT to determine the extent of the lesion, due to the high affinity of 67Ga for lymphoid tissue, 67Ga scan can be used as a supplementary examination to determine the extent of tumor invasion.
Diagnosis
Diagnosis and diagnosis of Hodgkin's disease in children
A complete diagnosis must include disease staging to guide clinical treatment and follow-up. Staged diagnosis can be made based on physical examination and related laboratory tests. The more common HD staging system is Ann Arbor staging.
Differential diagnosis
Painless progressive lymphadenopathy, especially in cervical lymphadenopathy, should be considered in this disease, but a biopsy is required to confirm the diagnosis, and should be distinguished from the following diseases:
Tuberculous lymphadenitis
Swelling lymph node biopsy is a reliable method of identification. Detailed medical history, tuberculin test and other sites (especially lung) can be used to identify the tuberculosis.
2. Acute lymphocytic leukemia
There may be significant local lymphadenopathy, but at the same time, there are often multiple lymph nodes of different degrees, and fever, anemia, hemorrhage, hepatosplenomegaly and other systemic symptoms are obvious, and more primitive lymphocytes in peripheral blood and bone marrow can be identified. .
3. Infectious mononucleosis
In addition to multiple sets of lymphadenopathy, more than 10% of atypical lymphocytes appear in the blood, and EBV-specific serological tests often have positive findings.
4. Malignant histiocytosis
There may be liver, spleen, and swollen lymph nodes, but the systemic symptoms such as fever, hemorrhage, and anemia are obvious. The disease progresses rapidly and often has liver, kidney and other organ damage. The number of white blood cells in the peripheral blood is often reduced. The bone marrow smear is found to be abnormal. Tissue cells can be identified, and individual atypical cases can only be identified by biopsy.
5. Benign lymphadenopathy
Such as massive lymph node hyperplasia or benign sinus histiocytosis with massive lymphadenopathy, such as benign lymphadenopathy, should be distinguished by clinical features and pathological biopsy.
6. Non-Hodgkin's lymphoma
HD has a slow onset and a long course of disease. It does not quickly endanger the life of the child. It starts from the lymph nodes, and the NHL in childhood is highly malignant. The course of the disease is short, which can often cause the child to be fatal quickly, and in addition to the lymph nodes. , often with bone marrow, testicular and central nervous system involvement, HD due to chronic progressive proliferation of lymphoid tissue to form a painless mass, lymphocytes and reticulocytes simultaneously proliferated, and visible RS cells for its characteristics, pathology and biopsy can be Identification.
7. Immunoblastic lymphadenopathy
Lymph node enlargement, fever, night sweats, weight loss and other symptoms similar to HD, lymph node biopsy can be identified.
