11/390. Chimeric monoclonal anti-CD20 antibody (rituximab)--an effective treatment for a patient with relapsing hairy cell leukaemia. A case story is presented, describing a 46 y old man, with a relapsing hairy cell leukaemia. After treatment with monoclonal anti CD-20 antibodies (rituximab) 375mg/week, four times, a complete remission was obtained which has lasted >9 months. The rituximab treatment produced a better remission than earlier treatments with alpha-interferon and chlorodeoxyadenosine. In addition, in contrast to other treatments, no initial worsening of the pancytopenia was observed. ( info) |
| This is the first report on a patient suffering from both multiple sclerosis (MS) and hairy cell leukemia. The patient was first treated with interferon-alpha. Due to disease progression two courses of cladribine were given resulting in an improvement of the clinical course of both diseases. Interestingly, it was possible to arrest and even ameliorate the progression of MS by administering as little as 30% of the dosage recently recommended for the treatment of this disease. ( info) |
13/390. Mycobacterium-avium-intracellulare complex infection following 2-chlorodeoxyadenosine therapy for hairy cell leukaemia. 2-Chlorodeoxyadenosine (2-CdA) is associated with prolonged suppression of CD4 lymphocytes. Cases of tuberculosis or mycobacterium avium intracellulare complex (MAC) infection complicating 2-CdA administration have not been reported despite the low CD4 counts. We report a patient with Hairy Cell Leukaemia (HCL) who developed MAC infection one month following 2-CdA treatment. This patient had been previously treated with prednisone for Sweets syndrome. It would appear that the combination of 2-CdA and prednisone predisposes patients to MAC infection, and if possible this combination of treatment should be avoided. ( info) |
14/390. Severe skin rash in two consecutive patients treated with 2-chlorodeoxyadenosine for hairy cell leukaemia at a single institution. Although hairy cell leukaemia was first described 40 years ago, it is only in the last decade that newer therapeutic agents have enabled effective treatment. The purine nucleoside analogue, 2-chlorodeoxyadenosine (2-CdA) is currently considered as first-line therapy with a very high rate of complete remission. Although adverse events with 2-CdA are increasingly recognized, severe cutaneous reactions have been reported rarely. We describe two consecutive patients treated with 2-CdA for hairy cell leukaemia who both suffered extremely severe cutaneous reactions, one of which was life-threatening. ( info) |
15/390. New rearrangement pattern after treatment of hairy-cell leukemia with 2-chlorodeoxyadenosine. Leukemic hairy cells are clonally proliferating B-lymphoid cells with clonal rearrangements of genes for immunoglobulin chains. We describe a patient with a new hairy-cell clone after treatment with 2-chlorodeoxyadenosine (2-CdA). In this patient, a single course of 2-CdA resulted in good partial remission of hairy-cell leukemia, but Southern blot analysis of bone marrow biopsies and polymerase chain reaction using seminested amplifications with consensus primers revealed a new rearranged band 4 months after therapy with 2-CdA. Four years after therapy, the patient is in complete clinical remission and both bands disappeared during follow-up. The new rearranged band might have been related to prior treatment of hairy-cell leukemia with 2-CdA. ( info) |
16/390. Co-existence of cutaneous T-cell lymphoma and B hairy cell leukemia. A primary cutaneous form of peripheral T-cell lymphoma (PTCL) and a low grade B-cell non-Hodgkin's lymphoma that was classified as a variant of hairy cell leukemia (HCL) were simultaneously diagnosed in a 79-year-old woman by both phenotypic and genotypic analyses. The coexistence of a T- and B-cell lymphoma in the same patient is rare, and, to our knowledge, this particular association has not been previously described. The patient was referred to our Department for evaluation of multiple cutaneous itchy, reddish plaques; laboratory analyses disclosed a lymphocytosis, that presented 6 years earlier. A bone marrow aspirate showed a 50% B-cell interstitial infiltrate, while a skin biopsy surprisingly revealed a PTCL. Clonality of both neoplastic processes was assessed by Southern blot analysis. The indolent clinical course of the cutaneous disease, and the low and stable number of circulating neoplastic T cells supported the diagnosis of a mycosis fungoides (MF)-like PTCL. Possible oncogenic events and/or putative underlying viral infections which could have played a role in the occurrence of B- and T-cell non-Hodgkin's lymphomas in the same patient are discussed. ( info) |
17/390. peliosis hepatis after treatment with 2-chloro-3'-deoxyadenosine. peliosis hepatis is an unusual disorder associated with a variety of diseases and treatments. This is the first report of peliosis hepatis associated with administration of 2-chloro-3'-deoxyadenosine. The literature is reviewed. ( info) |
18/390. Fatal cold anti-i autoimmune haemolytic anaemia complicating hairy cell leukaemia. Hairy cell leukaemia (HCL) is a rare lymphoproliferative disorder associated with pancytopenia, splenomegaly and the presence of typical hairy B lymphocytes in the bone marrow and/or peripheral blood. The most significant complication relates to opportunistic infections that arise as a consequence of neutropenia and monocytopenia. HCL is occasionally associated with systemic autoimmune disorders including polyarteritis nodosa and rheumatoid disease. Secondary autoimmune haemolytic anaemia (AIHA) appears to be rare. We report on two cases of HCL complicated by fatal cold anti-i AIHA. Fulminant haemolysis causing death is rare in cold AIHA and only a few individual cases have been reported, none having anti-i specificity. ( info) |
19/390. Structural and functional characteristics of hairy cells. Morphological, cytochemical, immunological and ultrastructural studies were performed on peripheral blood mononuclear cells from a patient with hairy-cell leukemia. Immunofluorescence studies showed a very strong intensity of fluorescence and indicated that hairy cells had monoclonal surface-membrane immunoglobulins (SmIg) actively produced by the cells. An unusual spontaneous SmIg redistribution induced by antibodies was also noted. Immunoultrastructural studies demonstrated that antibody-induced redistribution of SmIg on hairy cells is in form of a singular polar cap and that the cell membrane is rapidly cleaned of the complexes by endocytosis. The behavior of hairy cells regarding several membrane markers, mitogen stimulation and antibody-induced cytotoxicity suggests that hairy projections could represent the expression of a functional stage common to different lymphocyte subpopulations, or alternatively, a marker of a peculiar subset of B lymphocytes. ( info) |
20/390. Papillary cystic tumor of the pancreas coexisting with hairy cell leukemia. The coexistence of a pancreatic papillary cystic tumor with hairy cell leukemia is reported. To the best of our knowledge this association has never been published. A 41-year-old man diagnosed with hairy cell leukemia developed a second malignancy that corresponded to a papillary cystic pancreatic tumor. The patient underwent splenectomy and a tumoral surgical resection, and is currently well at 21 months follow-up. A pathogenetic relationship between the two malignancies was not demonstrated. Hairy cell leukemia has been reported to be associated to a great number of different second malignancies. In contrast, only two papillary cystic tumors of the pancreas have been described associated to a second neoplasm, a papillary thyroid carcinoma and a colonic carcinoma. This unusual benign or low-grade malignant pancreatic tumor more commonly occurs in the tail of the pancreas of young women. We want to stress the unusual presentation of this pancreatic tumor affecting the head of the gland in a male patient as well as its coexistence with a hairy cell leukemia. ( info) |