1/34. 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.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/34. 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.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
3/34. 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.- - - - - - - - - - ranking = 2.1325473372781keywords = neoplasm (Clic here for more details about this article) |
4/34. Atypical hairy cell leukemia.The morphologic differential diagnosis of mature B-cell neoplasms with cytoplasmic projections includes splenic lymphoma with villous lymphocytes and hairy cell leukemia. Although the classification of hairy cell leukemia is not universally recognized, 3 variants have been described, namely, classic, variant, and Japanese variant, each of which has different clinical and immunophenotypic features. Classic hairy cell leukemia is virtually always CD11c( ), CD25( ), and CD103( ). Variant and Japanese variant hairy cell leukemias are usually CD11c( ), always CD25(-), and occasionally CD103( ). Each variant is characteristically CD10(-). We present a case of hairy cell leukemia with a unique immunoprofile in that the cells were CD10( ), CD25( ), and CD103(-), and we review the criteria helpful in differentiating "hairy" B-cell neoplasms. This case emphasizes the variability of hairy cell leukemia and the need to correlate all clinical and pathologic data in reaching a diagnosis.- - - - - - - - - - ranking = 4.2650946745562keywords = neoplasm (Clic here for more details about this article) |
5/34. Simultaneous diagnosis of hairy cell leukemia and chronic lymphocytic leukemia/small lymphocytic lymphoma: a frequent association?The association of hairy cell leukemia (HCL) with other neoplasms, mainly non-Hodgkin's lymphomas, is well known. However, the simultaneous diagnosis of HCL and chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is rare, with only few cases of such an association having been reported. We describe three patients with a well-characterized HCL in whom a CLL/SLL population was detected. Of note, these cases represent a significant proportion (11.5%; 95% CI: 0% to 24%) of the total number of HCL cases diagnosed in our institution during the same period of time. All three patients were treated with deoxycoformycin. They achieved a complete response of the HCL, whereas the CLL/SLL population persisted in all cases. The immunoglobulin gene rearrangement analysis, in two informative cases, suggested that the HCL and CLL/SLL populations arose from different B cell clones. This study indicates that the association of HCL and CLL/SLL might be much more frequent than previously recognized. Therefore, a large panel of monoclonal antibodies, including those necessary to detect CLL/SLL, should be employed when studying patients with HCL.- - - - - - - - - - ranking = 2.1325473372781keywords = neoplasm (Clic here for more details about this article) |
6/34. Complex chromosomal rearrangements in an unusual variant of hairy cell leukemia.An unusual case of variant HCL with multiple ribosomal lamellar complexes and complex chromosomal changes is described. The karyotype of the main cell clone is characterized by involvement of chromosome 5 at q13.3 and interstitial deletion of 7q. However, there is no other evidence of myelodysplasia and the abnormal cells are of lymphoid origin with a B-cell immunophenotype. The clonal chromosomal evolution involves rearrangements at sites known to be specifically altered in lymphoid tumors.- - - - - - - - - - ranking = 0.4keywords = complex (Clic here for more details about this article) |
7/34. Ultrastructural modifications in one case of hairy cell leukemia during alpha-interferon therapy.There are many reports concerning the morphology of hairy cell leukemia (HCL), but, to our knowledge, there are no data on the ultrastructural modifications of HCL during interferon therapy. The ultrastructural modifications of neoplastic cells in peripheral blood in a case of HCL were investigated before and 2 and 4 months after beginning treatment with human lymphoblastoid alpha-interferon. Before therapy, hairy cells displayed the typical cytoplasmic projections, and 4% contained ribosome-lamellae complexes (RLC) (the cells contained up to 7 RLC). Two months from the beginning of therapy, hairy cells had shorter projections, RLC had disappeared, and tubuloreticular structures (TRS) had appeared in 2.2% of the elements. Four months from the beginning of therapy, TRS persisted in 2.3% of hairy cells, cylindrical confronting cisternae (CCC) appeared in 6.8% of the cells, and uncommon RLC, in close contact with the rough endoplasmic reticulum and nuclear membrane, were found in 1.5% of the elements. The cells contained up to 3 RLC. Our data confirm that interferon stimulates the synthesis of TRS and CCC, whereas the reappearance of uncommon forms of RLC could reflect their neosynthesis, possibly related to the interferon therapy. The frequent findings of a close contact between RLC and nuclear membrane support the view that RLC are derived not only from rough endoplasmic reticulum, but also from the nuclear membrane.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
8/34. Malignant histiocytosis with chronic course. Ultrastructural and ultrastructural cytochemical studies.Four cases of malignant histiocytosis with leukemic manifestations and chronic course were reported. light microscopic, ultrastructural and ultrastructural cytochemical details of these atypical cells were demonstrated. Ultrastructurally these cells resembled hairy cells most closely among the known varieties of leukemic cells. However, ribosome-lamella complexes were not found and some atypical cells had a few short cytoplasmic projections. In addition, tartrate-resistant acid phosphatase was absent from these cells. We speculate that this leukemic reticuloendotheliosis with a chronic course seen in japan seems to be analogous to malignant histiocytosis with massive splenomegaly reported by Vardiman et al.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
9/34. Epstein-Barr virus positive large B-cell lymphoma arising in a patient previously treated with cladribine for hairy cell leukemia.We describe the case of a patient treated with 2-chloro-2'-deoxyadenosine, CdA or cladribine for hairy cell leukemia who subsequently developed an Epstein Barr virus (EBV)-positive polymorphous large B-cell lymphoma (p-LBCL). The time interval between cladribine therapy and development of p-BCL was 11 months and morphologically resembled an EBV-positive post transplant lymphoproliferative disorder (PTLD). Molecular genetic studies for EBV-clonality by Southern blot hybridization showed a clonal population of infected cells, implying that this was an EBV induced lesion. The chronology of events suggest that cladribine, a purine analog which has been previously described to induce long-lasting immunodeficiency, can, in some cases, weaken the host defense mechanism to a level at which an innocuous EBV infection may transform the normal lymphoid cells into an aggressive neoplasm. Unlike most methotrexate-related lymphoproliferative disorders (LPDs), which undergo spontaneous remission after discontinuation of therapy, LPDs secondary to purine analogs often fails to resolve after discontinuation of therapy and requires additional therapy. Our patient was treated with rituximab following the diagnosis of p-LBCL, with the goal of improving the pancytopenia to permit chemotherapy. However, the patient failed to show any dramatic improvements in counts, developed systemic symptoms and progressive ascites. He expired 3 weeks after a second dose of rituximab. cladribine is a potent immunosuppressive agent and should be included with the list of immunosuppressive agents that may be associated with EBV-related B-cell lymphoproliferative disorders.- - - - - - - - - - ranking = 2.1325473372781keywords = neoplasm (Clic here for more details about this article) |
10/34. Successful treatment of extranodal Hodgkin's lymphoma in a patient with longstanding hairy cell leukemia.Purine analogs, particularly pentostatin and cladribine, are highly effective in hairy cell leukemia (HCL). Both of these drugs induce responses in approximately 80-95% of patients. However, it is not yet determined if treatment with these drugs can induce second malignancies. Hodgkin's lymphoma is very rare as a second malignancy and there are only 3 reported cases concerning the association of this lymphoma with HCL. We describe a patient with longstanding HCL in complete remission after cladribine, in whom extranodal Hodgkin's lymphoma appeared 8 years after the diagnosis of HCL. magnetic resonance imaging revealed diffuse intra-osseal neoplastic infiltration of the corpora of the whole spinal column and extra-osseal propagation from the fifth thoracic vertebra into the spinal canal with spinal cord compression. Histological and immunohistochemical analysis of the extradural tumor, which was completely excised, disclosed nodular sclerosis Hodgkin's lymphoma with typical reed-sternberg cells that were positive for CD30, CD15, bcl-6, Ki67, p53, EBV LPM-1 and IgG, and negative for CD45, CD20, DBA44, kappa, lambda light chains and IgM. In addition, immunohistochemical analysis of the bone marrow in 1999 showed infiltration with positivity for IgM and negative for kappa light chains and IgG. These findings (expression of different immunoglobulins and light chains on the cells) suggest an independent origin of these 2 B-cell neoplasms. After neurosurgery the patient received 6 courses of the MP-ABVD protocol and achieved a complete remission, which has lasted 16 months thus far.- - - - - - - - - - ranking = 2.1325473372781keywords = neoplasm (Clic here for more details about this article) |
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