Semi malignant cutaneous b cell lymphomas (SM-CBCL)
A M Jayaraman
F-40, TNHB Flats, No.4 Luz Church Road, Mylapore, Chennai-600 004.
|How to cite this article:
Dharmambal C, Ramesh A, Kumar P, Prabhavathy D, Jayaraman A M. Semi malignant cutaneous b cell lymphomas (SM-CBCL). Indian J Dermatol Venereol Leprol 2003;69:35-36
AbstractSemi-malignant CBCL, manifesting initially as chronic lymphatic leukemia in a 45 year old female is described.
Lymphoma, which signifies a malignant disease that can be controlled but not cured, and "Pseudolymphomas" which usually can be cured completely and remit by non-aggressive or aggressive treatment modalities. Semi malignant CBCL.1 a distrinct biologically benign subgroup based on clinical, biological, histological and phenotypical features, different from malignant CBCL because they do not tend to disseminate to extracutaneous sites or transform into high grade malignant blast type lymphomas.
45-year-old female admitted for asymptomatic skin lesions over face and neck for the past ten months. She gave history of systemic disturbances with skin lesions in the form of erythematous papules and plaques over face. Two years back she was diagnosed chronic lymphatic leukemia with appropriate investigation and had complete remission with Ch000lorambucil. But the skin lesions persisted for which no treatment intervention was done. Now for the past ten months she noticed fresh lesions over face and neck for which she was admitted. General examination was normal. Cutaneous examination revealed shiny tense erythematous papules and plaques of various sizes 3′2 cm - 2′1 cm over face and neck [Figure - 1]. No hypopigmented hypoanaesthetic patch. Peripheral verves were normal. Pathergy test was negative. On investigation, which included hemogram, peripheral smear, skiagram chest, ultra sonogram abdomen were normal. Mantoux test, serology for VDRL, ELISA for HIV, slit skin smear for AFB, Donovan bodies were negative. Patient was not cooperative for bone marrow biopsy. Skin biopsy (H&E) [Figure:2] showed normal epidermis with Grenz Zone and bottom heavy infiltrate showing dense diffuse lymphocytic infiltrate with atypicality. Phenotypically the cells expressed were CD-20, MB 2, and CD45R. Patient was referred to oncology department for further management.
According to REAL (Revised European American Lymphoma) classification, lymphoproferative disorders are divided into those of B-cell and T cell lineage and proliferation of hematolymphoid cells. Almost 50% of cutaneous B cell lymphoproliferative infiltrates in derived from follicular center cells. Among these, about 25% show a rapidly progressive course, whereas about 75% account for flatening of the survival curve after about 7 years. The latter group is referred to as SM-CBCL. Clinical, histological and phenotypical criteria for their differentiation from Bcell pseudolymphoma and CBCL are given in the table. Semimalignant CBCL differ from Pseudolymphoma in that complete cure of the usually multiple and disseminated skin manifestation is not possible and that follicular center formation and the network of CD21 positive cells in conjunction with a kappa or lambda light chain restriction of cellular surface immunoglobulins is seen. Based on past history of CLL (CBCL give leukemia picture in early stage), benignity (no extracutaneous involvement), normal blood smear study, positive phenotype marker, we fix the diagnosis of SM-CBCL for this patient whose prognosis is better than CBCL.
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