Name
CUTANEOUS T CELL LYMPHOMA
DESCRIPTION
DETAIL
CAUSES : UNKNOWN -------------------------------------------------------------------------- DIFFERENTIAL DIAGNOSIS: . Patches: . Parapsoriasis . Atopic eczema . Nummular eczema . Plaques: . Parapsoriasis . Psoriasis . Tumors: . Other primary skin cancers . Internal malignancies metastatic to skin . Histiocytosis X . Exfoliative erythroderma: . Psoriasis . Atopic dermatitis . Drug eruptionβ’ CBC with differential and platelets β’ Total lymphocyte count β’ Automated chemical profile SPECIAL TESTS : β’ SΓ©zary cell preparation β’ Electron microscopy β’ HTLV-l serology β’ Enzyme histochemistry β’ DNA flow cytometry β’ Immunophenotyping β’ Immunogenotyping β’ Molecular cytogenetics β’ Polymerase chain reaction IMAGING : β’ Chest x-ray β’ CT scan of abdomen DIAGNOSTIC PROCEDURES: β’ Skin biopsy - diagnostic procedure of choice β’ Lymph node biopsy β’ Bone marrow biopsy β’ Liver biopsy
TYPENOTES
GENERAL MEASURES : β’ No disease-specifi c special measures β’ Treatment should be individualized for each patient as in most cases this is a chronic slowly progressive disease. DRUG(S) OF CHOICE : . Therapy must be individualized. There is no universally accepted standard approach to treatment of this disease. The extent and the rapidity of progression of the disease (stage of disease) dictates the aggressiveness of therapy. 1. For early and less aggressive disease: . Topical potent corticosteroids . Topical mechlorethamine (nitrogen mustard, Mustargen) . PUVA or RePUVA (retinoids plus PUVA) . Topical carmustine (BCNU) 2. For more resistant disease: . Electron beam therapy . Combination therapy - interferon or retinoids plus phototherapy (UVA or UVB) . Methotrexate . Extracorporeal photochemotherapy (especially Sezary syndrome) 3. For advanced and resistant disease: . Systemic chemotherapy . Interleukin-2 . Bexarotene (Targretin) . Topical mechlorethamine (nitrogen mustard, Mustargen) for early disease, in aqueous or ointment preparation applied once daily to entire skin surface avoiding genitalia ALTERNATIVE DRUGS : β’ Narrow band or broad band UVB β’ PUVA (psoralen and ultraviolet A light) β’ Topical carmustine (BCNU) β’ Total body electron beam therapy β’ Localized orthovoltage x-ray therapy β’ Interferon β’ Retinoids (e.g., etretinate) β’ Extracorporeal photochemotherapy β’ Denileukin diftitox (Ontak) β’ Purine analogues (deoxycoformycin, fludarabine, and 2-chlorodeoxyadenosine) β’ Bone marrow transplantation PATIENT MONITORING: Must be individualized POSSIBLE COMPLICATIONS : Metastatic spread to organs other than skin EXPECTED COURSE/PROGNOSIS : β’ The development of lymphadenopathy, tumors or cutaneous ulcerations during the course of the disease is associated with a median survival of 4 years β’ The appearance of the third sign (irrespective of the order developed) is associated with a median survival of 1 year
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
X-RAY CHEST P.A. VIEW( NORMAL ), COMPLETE BLOOD COUNT, CT SCAN ABDOMEN, BIOPSY