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Yibian
 Shen Yaozi 
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diseaseChildhood Hodgkin's Disease
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bubble_chart Overview

Hodgkin's disease (HD) is caused by chronic progressive malignant proliferation of lymphoid tissue, characterized by progressive enlargement of cervical lymph nodes and the presence of Hodgkin's cells (R-S cells) in pathological components. The treatment prognosis is relatively favorable.

bubble_chart Diagnosis

(1) Clinical Manifestations 1. Systemic symptoms: Fever, night sweating, loss of appetite, etc. 2. Primary follicular tumor manifestations: - 60–90% of pediatric cases present with primary follicular tumors in the cervical lymph nodes, characterized by painless, progressive enlargement that may coalesce into masses. - Approximately 1/3 to 2/3 of cases exhibit mediastinal masses. - Initial involvement of axillary or inguinal lymph nodes is rare. - Primary lesions below the diaphragm are uncommon. - A minority present with generalized lymphadenopathy accompanied by periodic high fever. 3. Local compressive symptoms: May include cough, dyspnea, Horner's sign, hoarseness, and aphasia. 4. Symptoms of extranodal organ infiltration. (2) Laboratory and Auxiliary Examinations

  1. **Blood tests**: Normal or anemia may be observed. Differential white blood cell counts may reveal eosinophilia and monocytosis, with Reed-Sternberg (R-S) cells visible in a minority of cases.
  2. **Bone marrow examination**: In advanced stages, R-S cells may be detected. These cells are large (approximately 15–80 µm), multinucleated, and typically feature two mirror-image nuclei with large, deep blue "owl's eye" nucleoli.
  3. **Imaging studies**: Ultrasound, X-ray, CT, and, when necessary, bone, liver, or spleen radionuclide scans aid in diagnosing tumor foci in the chest, abdomen, or skeletal system.
  4. **Biopsy**: Histopathological examination is the primary method for diagnosing Hodgkin's disease (HD). Lymph node sections or smear preparations of aspirated fluid show a mixture of inflammatory-reactive components and neoplastic R-S cells. Identification of R-S cells confirms the diagnosis. Liver, spleen biopsies, or tumor tissue sampling may be performed during seasonal epidemics if needed. Based on histocytological changes, HD is classified into: (1) Nodular sclerosing type; (2) Lymphocyte-predominant type; (3) Mixed cellularity type; (4) Lymphocyte-depleted type. In children, types (1) and (2) are common and carry a favorable prognosis, while type (4) is rare and has the poorest prognosis. (3) Clinical Staging Diagnosis - **Stage I**: Disease confined to a single lymph node or lymph node region (I) or a single extranodal organ (IE). - **Stage II**: Disease involves two or more lymph node groups on the same side of the diaphragm (II) or a localized organ with involvement of one or more lymph node groups on the same side (IIE). - **Stage III**: Lymph node regions on both sides of the diaphragm are involved (III), possibly accompanied by localized extranodal organ involvement (IIIE) or splenic invasion (IIIS). - **Stage IV**: Widespread or disseminated involvement of one or more extranodal organs, with or without lymphadenopathy. Each stage is further subdivided into groups A and B: - **A**: Absence of fever, weight loss (≥10%), or night sweating. - **B**: Presence of any of the above symptoms.

bubble_chart Treatment Measures

﹝Treatment﹞

(1) Treatment Principles for Different Disease Stages Ⅰ, Ⅱ stages: Select MOPP or ABVD or alternate between the two for 6 cycles of chemotherapy as appropriate. Low-dose (25Gy or 15–25Gy) involved-field radiotherapy is administered after the 3rd cycle or upon completion of 6 cycles. The 5-year disease-free survival (DFS) rate exceeds 90% and can reach 100%. For stage ⅠA or ⅡA without bulky tumors, radiotherapy may be omitted. Patients with bulky mediastinal masses or B symptoms must undergo combined modern chemotherapy and localized radiotherapy. ⅢA stage: Combined chemotherapy for 8–12 months, with localized radiotherapy added as appropriate. ⅢB, ⅢS, and Ⅳ stages: Combined chemotherapy includes induction remission, consolidation, and maintenance therapy, with a total treatment duration of 2 years. Localized radiotherapy may be added as appropriate. DFS can reach 60–90%. (2) Common Chemotherapy Regimens MOPP regimen: M (Mechlorethamine) 6mg/(m²·dose) IV, days

  1. and 8. O (Vincristine) 1.4mg/(m²·dose) IV, days 1
and 8. P (Procarbazine) 100mg/(m²·day) PO, days 1–15. P (Prednisone) 40mg/(m²·day) PO, days 1–15. Repeat every 28 days. ABVD regimen: A (Doxorubicin) 25mg/m² IV, days
  1. and 15. B (Bleomycin) 10mg/m² IV, days 1
and 15. V (Vincristine) 6–10mg/m² IV, days
  1. and 15. D (Dacarbazine) 250–375mg/m² IV, days 1
and 15. Repeat every 28 days. In recent years, alternating between MOPP and ABVD regimens has been advocated to avoid or reduce the toxic side effects of continuous use of a single regimen, while also improving efficacy. If the above regimens yield unsatisfactory results, the following alternatives may be considered: ChlVPP regimen: Chlorambucil and vincristine replace mechlorethamine and vincristine in MOPP. CVPP regimen: Cyclophosphamide and vincristine replace mechlorethamine and vincristine in MOPP. DPPA regimen: Anthracyclines (e.g., pirarubicin) replace mechlorethamine in MOPP. These regimens are alternated with ABVD, etc. Recently, reports from abroad have shown promising results in treating refractory and relapsed Hodgkin's disease (HD) with high-dose chemotherapy combined with hematopoietic growth factors and hematopoietic stem cell transplantation.

bubble_chart Differentiation

Attention should be paid to differentiation from subcutaneous lymph nodes, histiocytosis, infectious mononucleosis, immunoblastic lymphadenopathy, necrotizing lymphadenitis, and others.

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