title: [Leg ulcer associated with type I cryoglobulinaemia due to incipient B-cell lymphoma].
abstract: BACKGROUND
  Skin lesions are frequent in monoclonal cryoglobulinaemia and may be the first sign of B-cell lymphoma, especially multiple myeloma, Waldenström's macroglobulinaemia and B-cell chronic lymphocytic leukaemia.
  
  
  METHODS
  A 74-year-old woman with no prior medical history presented with necrotic leg ulcer. Skin biopsy showed dermal angiomatosis with numerous PAS+ thromboses, associated with monoclonal intravascular deposits of IgM kappa, indicating monoclonal cryoglobulin, which was confirmed by laboratory tests. Subsequent blood immunophenotyping revealed an inconspicuous circulating monoclonal CD5(+) B-cell population and small B-cell clusters in the bone marrow, while the B-cell count was normal and no lymphadenopathy or splenomegaly were present. Overall, these findings indicated a small B-cell lymphoma, classed as non-MALT marginal zone lymphoma on the WHO classification, at a very early stage of development. The patient was first treated by cyclophosphamide and oral steroids without success. Subsequent administration of six cycles of rituximab, cyclophosphamide, vincristine and prednisone (RCVP) led to remission of her leg ulcer, cryoglobulinaemia and lymphoma.
  
  
  CONCLUSIONS
  Skin biopsies of necrotic ulcers should undergo routine screening for intravascular deposits of type 1 cryoglobulin. Leg ulcers due to monoclonal cryoglobulinaemia may reveal incipient marginal zone B-cell lymphoma at the stage of circulating monoclonal lymphocytosis.
authors.affiliation: Département de pathologie, Inserm U955 équipe 9, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris (AP-HP), faculté de médecine, université Paris Est Créteil-Val-de-Marne, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94010 Créteil cedex, France.
authors.forename: C
authors.initials: C
authors.lastname: Lacoste
authors.forename: T A
authors.initials: TA
authors.lastname: Duong
authors.forename: J
authors.initials: J
authors.lastname: Dupuis
authors.forename: C
authors.initials: C
authors.lastname: Haioun
authors.forename: A
authors.initials: A
authors.lastname: Plonquet
authors.forename: C
authors.initials: C
authors.lastname: Copie-Bergman
authors.forename: P
authors.initials: P
authors.lastname: Gaulard
authors.forename: N
authors.initials: N
authors.lastname: Ortonne
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000737:chemistry / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D003449:Cryoglobulinemia / Q000209:etiology*; D003450:Cryoglobulins / Q000032:analysis; D003520:Cyclophosphamide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D016130:Immunophenotyping; D007871:Leg Ulcer / Q000209:etiology* / Q000473:pathology; D018655:Lymphocyte Count; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D011241:Prednisone / Q000008:administration & dosage; D012074:Remission Induction; D000069283:Rituximab; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000305:Adrenal Cortex Hormones; D058846:Antibodies, Monoclonal, Murine-Derived; D003450:Cryoglobulins; D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains; D000069283:Rituximab; D014750:Vincristine; D003520:Cyclophosphamide; D011241:Prednisone