1/34. Hairy cell leukemia in first cousins and review of the literature.Familial hairy cell leukemia (HCL) occurs rarely. So far, 26 familial instances of HCL (in 12 families) have been reported in the literature. The consistent human leukocyte antigen (HLA) linkage could not be established in most cases of familial HCL. history of exposure to organic chemicals or employment in woodworking or farming was noted in only two out of 11 affected families. We present two familial cases of HCL as well as a thorough literature review. An influence of HLA or farming themselves on a predisposition to HCL remains unproven but does not rule out an HLA-linked and as yet unidentified gene responsible for increased disease susceptibility. HCL in families is unlikely to be due to random patterning, but there are insufficient data so far to decisively incriminate either HLA-related or environmental causative factors.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/34. Rituximab-induced acute thrombocytopenia: a report of two cases.Rituximab use in B-cell malignancies has been widely favored by the acceptable toxicity profile of this drug. Episodes of rituximab-induced neutropenia have been reported in some patients, but severe acute thrombocytopenia is very unusual. Here, we report transient severe acute thrombocytopenia after rituximab infusion in two patients with, one hairy cell leukemia the other with mantle cell lymphomay. Interestingly, in both cases, thrombocytopenia was reversible in a few days without further therapeutic intervention. The mechanism of this side effect remains unclear. Previous reports suggested the presence of CD20 antigen on the platelets themselves or that soluble CD20 antigen in the circulation may cause an antigen-antibody reaction and immune-mediated cell lysis. It is noteworthy that the two cases reported here as well as the two previously published cases share massive bone marrow involvement by neoplastic B lymphocytes.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
3/34. Ki-1-positive lymphoma developing 10 years after the diagnosis of hairy cell leukemia.The case is described of a 62-year-old man with a 10-year history of hairy cell leukemia (HCL) who subsequently had a large-cell anaplastic or so-called Ki-1-positive lymphoma. Immunocytochemical staining of the lymphomatous node revealed positivity for Ki-1 (CD30) and epithelial membrane antigen in the tumor cells, and flow cytometric analysis showed simultaneous expression of Leu M5 (CD11c) and Leu 14 (CD22). Although HCL has been reported to coexist with both Hodgkin's disease and non-Hodgkin's lymphoma, the authors believe this is the first case in which a Ki-1-positive lymphoma developed in a patient with HCL. The clinicopathologic and immunologic features of both entities are discussed, as is the association of HCL with other neoplasms.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/34. sezary syndrome in a patient with hairy cell leukemia in remission.A 65-year-old man was evaluated for pancytopenia in March 1979, and found to have hairy cell leukemia (HCL). Treatment with splenectomy and subsequently interferon produced temporary remissions. In July 1985, the patient began intravenous deoxycoformycin (DCF) therapy, and after 1 year complete peripheral blood and bone marrow remission was achieved. Fourteen months after cessation of therapy, the patient developed a skin rash and was found to have cutaneous T-cell lymphoma and sezary syndrome. Morphologic study of the hairy cells (HC) in the peripheral blood at presentation and the Sezary cells was distinct by light and electron microscopic study. immunophenotyping of peripheral blood mononuclear cells showed clearly that the HC were of B-cell origin (CD20 , sIg ), whereas the lymphoid population at second presentation was T-cell (CD3 , CD4 , HLA-DR-). Clonal rearrangement of T-cell antigen receptor beta-chain gene was detected by Southern analysis of the Sezary cell population, whereas immunoglobulin heavy and light chain genes remained in germ line configuration. This is the first case of sezary syndrome developing in a patient previously treated for HCL where studies have confirmed distinct B-cell and T-cell origin of the two neoplasms. The authors suggest that treatment and disease-related immunosuppression are possible etiologic factors in the development of this second lymphoid neoplasm.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/34. HTLV-I associated myelopathy (HAM) after blood transfusion in a patient with CD2 hairy cell leukemia.Hairy cell leukemia complicating hemolytic anemia developed in a 46-year-old woman. Morphologically and cytochemically typical hairy cells were found to express both CD20 and CD2 antigens. Expression of surface IgG of kappa-chain type and the rearrangement of Ig but not T-cell receptor beta genes confirmed a B-cell origin of the leukemia. blood transfusion was followed by disappearance of the hemolysis and a marked improvement of the leukemia. However, the patient developed progressive spastic spinal paraplegia about seven months after transfusion and was diagnosed as having HTLV-I associated myelopathy (HAM) by the demonstration of htlv-i antibodies in serum and cerebrospinal fluid. HTLV-I infection via the transfusion may have been involved in the hematologic improvement seen in this patient. autopsy showed demyelination, vacuolar degeneration, gliosis, and perivascular cuffing in the white matter of spinal cord without evidence of leukemic infiltration.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/34. Hairy cell leukemia-variant--a case report.Hairy-cell leukemia-variant (HCL-V) is a rare B-cell disorder which accountsfor 10% of HCL cases. The main features are splenomegaly, lymphocytosis and cytopenias without monocytopenia. The circulating cells have a morphology intermediate between prolymphocytes and hairy cells. The immunophenotype shows a mature B-cell phenotype with expression of B-cell antigens CD11c and CD103 but unlike typical hairy cell the cells are negative for CD25. The histology of bone marrow and spleen shows a pattern of infiltration similar to that in HCL. We present a case of HCL-V in a 66-year-old male. The bone marrow findings, immunophenotypic profile and electron microscopic features are described. The patient underwent splenectomy which also revealed infiltration by leukemia. patients are resistant to alkylating agents and alpha-interferon (a-IFN). splenectomy may be beneficial for long-lasting partial responses in some of the patients and is a good palliative treatment.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/34. Dual rearrangement of immunoglobulin and T-cell receptor gene in a case of T-cell hairy-cell leukemia.We report a case of T-cell hairy-cell leukemia with a dual rearrangement of Ig- and T-cell receptor genes. The cytochemical, transmission electron microscopy, and surface antigens data (CD3 , CD8 , CD11 , HLA-DR , CD19-, CD20-) were consistent with a T-cell hairy-cell leukemia. Molecular analysis according to Southern revealed a dual rearrangement of immunoglobulin heavy-chain (JH) and T-cell receptor beta (TcR beta) chain genes. Our findings suggest that the coexistence of JH and TcR gene rearrangements, frequently detected in acute leukemia, may also be observed in hematologic malignancies derived from more differentiated cells.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/34. Hairy cell leukemia in two siblings. A human leukocyte antigen-linked disease?Shared haplotypes have been documented in three of the four previously reported families with familial hairy cell leukemia (HCL). Human leukocyte antigen (HLA) typing was done on a four-member sibship in which two siblings had hairy cell leukemia. The two affected siblings share the haplotype A2; Bw6, Bw62(15); Cw1; DR4, DRw53; DQw3. No one HLA antigen or haplotype is common among the five families reported at this time. These limited data suggest HCL is not associated with a specific HLA antigen. HLA typing of a larger collection of cases of familial HCL may help better define what role, if any, inheritance plays in the occurrence of this rare disorder among numerous first degree relatives.- - - - - - - - - - ranking = 7keywords = antigen (Clic here for more details about this article) |
9/34. Establishment of a hairy cell leukemia cell line carrying Tac antigen and phagocytic activity with B-cell characteristics.A hairy cell leukemia cell line designated "hair-M" was established in a suspension culture derived from the peripheral blood of an 86-year-old Japanese male with a diagnosis of hairy cell leukemia. The hair-M cells were identified as having prominent hair-like cytoplasmic projections by examination with phase-contrast and scanning electron microscopy. These cells displayed ruffled membranes and stublike microvilli similar to those observed on the surfaces of cells in the peripheral blood of the patient. Immunologic and cytochemical studies on the hair-M cells confirmed derivation from the clone of the patient's leukemia cells. Although the cultured hair-M cells had definite B-cell characteristics, such as IgG kappa-chains on the surface and in cytoplasm, they also demonstrated Tac antigen, which is usually expressed on activated T-cells, and myelomonocyte antigens determined by OKM-1 and MCS-1 monoclonal antibodies. Other cell surface markers, including E(-), IgGFc(-), IgMFc(-), C3R( ), Ia-like antigen( ), OKT9( ), OKT10( ), and terminal deoxynucleotidyl transferase(-), were detected; no Epstein-Barr virus-determined nuclear antigen was detected. The karyotype of the hair-M cells was determined to be 46XY with -11, -14, and two marker chromosomes. The hair-M cells also had phagocytic activity to rabbit anti-human IgG serum-coated polyacrylamide gel particles.- - - - - - - - - - ranking = 8keywords = antigen (Clic here for more details about this article) |
10/34. Morphological and immunological changes of hairy cell leukemia during alpha-2-interferon therapy.We describe a patient who presented with the clinical picture of hairy cell leukemia (HCL). bone marrow and peripheral blood lymphoma cells showed morphological and immunological features of HCL. Under recombinant alpha-2-interferon (alpha-2-IF) therapy the characteristic morphology changed from HCL to prolymphocytic leukemia (PLL). At diagnosis the lymphoma cells expressed CD24 and FMC7 surface antigen, but stained negative for surface immunoglobulins, light chains and anti-CD5. During alpha-2-IF treatment surface antigen expression changed to CD24, CD5 and FMC7. Surface IgD and lambda light chains became strongly positive. Southern Blot analysis of peripheral blood mononuclear cells showed two rearranged immunoglobulin bands at diagnosis but only one upon alpha-2-IF therapy. These data suggest, that this patient suffered from a biclonal lymphoma, HCL and PLL. While undergoing alpha-2-IF treatment the HCL came into remission, whereas the PLL clone proved to be poorly sensitive to alpha-2-IF therapy.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
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