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Castleman s Disease: a case study

Castleman's Disease: a case study. Hematology/Oncology Grand Rounds November 8, 2002Shao-Chun Chang. History. 35-year-old healthy man until July 8, 2002Sharp, intense right flank and back pain with radiation to groinGross hematuriaNausea and vomitingNo constitutional Sx, no neuro Sx, no other pain, no other GU Sx, no HIV risk factorsNo PMHxPE: RLQ mass with guarding without rebound.

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Castleman s Disease: a case study

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    2. Castleman’s Disease: a case study Hematology/Oncology Grand Rounds November 8, 2002 Shao-Chun Chang

    3. History 35-year-old healthy man until July 8, 2002 Sharp, intense right flank and back pain with radiation to groin Gross hematuria Nausea and vomiting No constitutional Sx, no neuro Sx, no other pain, no other GU Sx, no HIV risk factors No PMHx PE: RLQ mass with guarding without rebound

    4. Laboratory CBC normal without cytopenia and normal indicies and differential Chemistry normal Urine positive for RBC

    5. Radiology Initial workup for nephrolithiasis IVP showed effacement of bladder secondary to a pelvic mass CT of abdomen/pelvis 7.8 x 5 cm mass in right pelvis Enlarged mesentery and inguinal lymph nodes Mild right hydronephrosis and hydroureter

    6. Additional Laboratory LDH normal SPEP Polyclonal hypergammaglobulinemia Small M-spike in beta region (~0.2 g/dl), IgA lambda Beta2-microglobulin normal AFP normal, HCG normal HHV8 negative, HIV negative

    7. Pathology Cervical and inguinal biopsy Chronic reactive lymphoid hyperplasia Pelvic mass biopsy Angiofollicular lymphoid hyperplasia, plasma cell variant Flow cytometry showed polyclonal T and B cell populations

    8. Staging Bone marrow biopsy was non-diagnostic PET scan Uptake to the right pelvic mass and regional lymph nodes consistent with CT No distant uptake Exploratory Laparotomy Unresectable pelvic mass encasing the IVC and right iliac artery and vein Right ureter stent placement

    9. Castleman’s Disease Angiofollicular lymphoid hyperplasia Giant lymph node hyperplasia Angiomatous lymphoid hamartoma Lymph nodal hamartoma Lymph node hyperplasia of Castleman

    10. Castleman’s Disease First described by Benjamin Castleman “Localized mediastinal lymph-node hyperplasia resembling thymoma” Cancer (1956), 9:822-830 “A series of thirteen cases of mediastinal masses resembling thymoma grossly and microscopically are shown to be a peculiar form of lymph-node hyperplasia characterized by germinal-center formation and marked capillary proliferation.” “Evidence is presented that the condition is neither neoplastic nor thymic in origin.”

    11. Angiofollicular Lymphoid Hyperplasia

    12. Castleman’s Disease: Eitology Unknown Reactive chronic lymphoid hyperplasia (Hyaline-vascular type) Inflammatory pathogensis (Plasma-cell type) Chronic antigenic stimulation (infection) Autoimmune mechanism Overproduction of IL-6 Association with HHV8 in 25% multicentric form HIV?

    13. Castleman’s Disease Keller, AR, Hoghholzer, L, and Castleman, B, Cancer (1972), 29:670-683 Case study of 81 patients All had localized disease Two distinct histologic patterns Hyaline-vascular type: 74 cases (91%) Plasma-cell type: 7 cases (9%) Age: 8- to 66-years-old No gender or race preference

    14. Castleman’s Disease: Anatomical Location

    15. Castleman’s Disease: Anatomical Location

    16. Angiofollicular Lymphoid Hyperplasia: Hyaline-Vascular Type Small follicle center with penetrating capillaries Interfollicular tissue with small lymphocytes Effacement of lymphoid sinsus

    17. Angiofollicular Lymphoid Hyperplasia: Plasma-Cell Type Large cellular follicle center Interfollicular tissue with sheets of plasma cells Effacement of lymphoid sinsus

    18. Castleman’s Disease: Multicentric Histopathologic features of plasma-cell type Clinical presentation as a predominantly lymphadenopathic disease, consistently involving multiple peripheral nodes Evidence of multisystem involvement Idiopathic

    19. Castleman’s Disease: Clinical Symptoms Hyaline-vascular type (localized) Asymptomatic, except for tumor compression Plasma-cell type (localized) +/- Systemic symptoms Plasma-cell type (multicentric) By definition with systemic symptom +multisystem involvement

    20. Multicentric Castleman’s Disease: Clinical Symptoms System symptoms (95%) Malaise 81% Fever 71% Weight loss 58% Night sweats 48% Anorexia, nausea 42% Multicentric LAD (100%) Peripheral 100% Abdomen 53% Mediastinum 47% Splenomegaly 79% Hepatomegaly 63% Skin rashes 37% Neurological Sx Central 24% Peripheral 5% Kaposi’s sarcoma 13% NHL 18%

    21. Multicentric Castleman’s Disease: Laboratory Anemia 89% Mild (10-13 g/dL) 27% Moderate-severe (<10 g/dL) 73% Leukopenia 21% Thrombocytopenia 61% Elevated ESR 95% Hypergammaglobulinemia 85% Hypoalbuminemia 100% Abnormal liver tests 69% Proteinuria 83%

    22. Castleman’s Disease: Prognosis and Treatment Hyaline-vascular type Prognosis good Surgical resection Plasma-cell type, localized Prognosis good Curable with local therapy (surgery or radiation)

    23. Castleman’s Disease: Prognosis and Treatment Plasma-cell type, multicentric Unpredictable course (medium survival of 29 months) Cause of death: 70% infection and 30% others (malignancies, progressive Castleman’s disease, renal failure) Surgical resection Radiation High dose steroids (1-2 mg/kg/d prednisone) Combination chemotherapy (NHL) Anti-IL-6 antibodies

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