Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Crystal storing histiocytosis (CSH) is a rare condition where crystals accumulate in the cytoplasm of macrophages and is usually associated with a lymphoplasmacytic neoplasm producing a monoclonal immunoglobulin with kappa light chain. CSH with primary manifestation in the skin is extraordinarily rare and limited to four case reports in the literature. Here we present two cases of cutaneous CSH. One case is that of an 80 year old woman who presented with bilateral periorbital oedema with yellow discolouration. Skin biopsy showed dermal CSH in association with a neoplastic lymphoplasmacytoid infiltrate showing IgM kappa light chain restriction. Subsequent work up led to the discovery of Waldenstrom's macroglobulinemia. She had an indolent clinical course but died 6 years later from transformation into a diffuse large B cell lymphoma in the bone marrow. The other case is that of a 52 year old man who was recently diagnosed with multiple myeloma and developed a pruritic rash on his back during chemotherapy. Skin biopsy showed Grover disease (transient acantholytic dermatosis) and crystal storing macrophages in the superficial dermis. He died 4 years later after a protracted clinical course involving multiple cycles of chemotherapy and numerous complications. In both patients cutaneous CSH occurred early in the course of their lymphoplasmacytic malignancy and its development did not herald rapid clinical decline.
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PMID:Cutaneous crystal storing histiocytosis: a report of two cases. 2535 27

Primary cutaneous follicle center lymphoma (PCFCL) is the most common type of cutaneous B-cell lymphoma. The cutaneous manifestations of PCFCL typically include solitary erythematous or violaceous plaques, nodules, or tumors of varying sizes. Grouped lesions also may be observed, but multifocal disease is rare. We report the case of a 54-year-old woman diagnosed with PCFCL with an unusual clinical presentation resembling folliculitis or Grover disease. Histologic studies demonstrated extensive lymphoma cells in a nodular and diffuse pattern. Immunohistochemical studies demonstrated that the neoplastic cells were positive for CD20, CD79a, BCL-2, and BCL-6; CD3, CD5, and cyclin D1 were negative. These findings were consistent with PCFCL. Further evaluation for systemic disease via positron emission tomography-computed tomography and bone marrow biopsy was unremarkable. Increased awareness of this presentation of PCFCL can facilitate earlier diagnosis and intervention, which may result in improved patient outcomes.
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PMID:Primary cutaneous follicle center lymphoma mimicking folliculitis. 2952 19