1/13. Severe pancytopenia and hemophagocytosis after HHV-8 primary infection in a renal transplant patient successfully treated with foscarnet.We report the occurrence of human herpesvirus (HHV)-8 primary infection in an adult male kidney recipient. Four months after transplantation, the patient developed visceral Kaposi sarcoma, and 1 month later he presented with progressive and severe peripheral cytopenia, in the presence of a normocellular or hypercellular bone marrow (BM) with hemophagocytosis. HHV-8 was the sole pathogen detected by polymerase chain reaction either in the serum or in the BM. HHV-8 latent nuclear antigen was detected in immature progenitor cells from the BM. Immunosuppressive therapy was reduced, and the patient was treated with foscarnet for 2 weeks, leading to a dramatic normalization of blood cell counts, concomitantly with the disappearance of HHV-8 viremia. At the end of antiviral therapy, the patient received chemotherapy, and Kaposi sarcoma regressed in 2 months. Severe peripheral cytopenia may be a posttransplant complication after HHV-8 infection, for which treatment with foscarnet seems appropriate.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/13. pancytopenia due to suppressed hematopoiesis in a case of fatal hemolytic disease of the newborn associated with anti-K supported by molecular K1 typing.The authors report on a fatal case of hemolytic disease of the newborn (HDN) due to anti-K antibodies with subsequent trilineage pancytopenia in a preterm infant of 28 weeks gestational age, with pronounced leukopenia and neutropenia. In addition, molecular typing of the Kk polymorphism was necessary to confirm HDN. This case of HDN associated with anti-K provides additional evidence that trilineage pancytopenia due to suppressed hematopoiesis is part of the disease. Therefore, antibodies against antigens of the Kell blood group system should be considered as a potential cause of unexplained inhibition of myelopoiesis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/13. Remarkable response to rituximab in a patient with atypical CD20( ) mantle cell lymphoma of the bone marrow leading to severe pancytopenia.Rituximab, a chimeric monoclonal antibody which binds to the CD20 antigen, has been reported in several studies to induce remissions in low- and high-grade non-Hodgkin's lymphoma without causing myelosuppression. We report here a case of a 68-year-old female patient with an atypical mantle cell lymphoma infiltrating only the bone marrow without leukemic involvement or any other nodal or extranodal manifestations. Progressive severe pancytopenia due to the diffuse bone marrow infiltration led to life-threatening infections following oral chlorambucil treatment. No response to chlorambucil was noted. The patient attained a complete remission after salvage therapy with four weekly infusions of single-agent rituximab at a standard dose of 375 mg/m(2). Thus, anti-CD20 antibody may be the treatment of choice for patients with CD20( ) B-non-Hodgkin's lymphoma who cannot tolerate chemotherapy due to high risk of infectious complications as a result of severe pancytopenia.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/13. Development of hepatosplenic gammadelta T-cell lymphoma with pancytopenia during early pregnancy: a case report and review of the literature.Lymphomas rarely develop during pregnancy, but hepatosplenic gammadelta T-cell lymphoma (HSgammadeltaTCL) is extremely rare. We encountered a case of T-cell intracellular antigen-1 (TIA-1) positive and granzyme B-positive HSgammadeltaTCL that developed early during the course of pregnancy. The patient was a 31-yr-old female who was referred to our hospital because of pancytopenia and splenomegaly at the time of the14th week of her gestation. Her pancytopenia and hepatosplenomegaly worsened and she became fibril at the 27th week of gestation and cesarean section was performed at the 29th week. Histopathological examination of the spleen, which was resected 28 d after delivery for a diagnostic purpose, revealed medium to large-sized nodules composed of dense proliferation of lymphoid cells having round to oval-shaped nuclei and abundant weakly eosinophilic cytoplasm. They were CD3epsilon , mCD3 , CD4-, CD8-, CD56 , CD79a-, T-cell receptor (TCR)-gammadelta protein , TIA-1 , and granzyme B by either immunohistochemistry or flow cytometry. Clonal rearrangement of TCR-gamma genes without such rearrangement of TCR-delta and TCR-beta genes was confirmed by Southern blot hybridization. Thus, the patient was diagnosed as having HSgammadeltaTCL, and combination chemotherapy was initiated. She is currently in partial remission. To our knowledge, this is the first case report of HSgammadeltaTCL that developed during pregnancy. Pathogenesis of pregnancy-associated lymphoma is not known, but it is possible that maternal immunity during pregnancy or a hormonal imbalance, such as a change in the progesterone level, induces the development of lymphoma. pregnancy-associated lymphoma is resistant to standard chemotherapy and is associated with poor prognosis. Therefore, it is important to accumulate clinicopathologic data of such cases for the development of a treatment modality.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/13. vincristine therapy for severe platelet alloimmunization.A 19-month-old girl with idiopathic severe aplastic anemia refractory to multi-agent immunosuppressive therapy developed severe platelet alloimmunization following several months of platelet transfusions. She became refractory to human leukocyte antigen (HLA)-matched platelet transfusions and experienced frequent episodes of bleeding. She was treated with intravenous vincristine administered weekly for three doses and showed marked improvement in both clinical and laboratory response to platelet transfusions. When vincristine was held for 3 weeks, she again became refractory to HLA-matched platelet transfusion. Reinstitution of vincristine resulted in cessation of clinical bleeding and improved response to platelet transfusion. The mechanism of response likely involves selective delivery of cytotoxic drug to macrophages. To our knowledge this is the first reported case of alloimmune thrombocytopenia responsive to vincristine.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/13. Simultaneous primary infection with HIV and CMV leading to severe pancytopenia, hepatitis, nephritis, perimyocarditis, myositis, and alopecia totalis.Simultaneous primary infection with HIV and CMV in an 18-year-old woman led to an acute cytotoxic reaction, manifesting as pancytopenia, hepatitis, nephritis, perimyocarditis, and myositis. Within 14 days parameters indicating acute cell damage reverted to normal. Two weeks later transient alopecia totalis developed. Initially, HIV-antigen but no hiv antibodies were present. Within 3 weeks HIV-IgG antibodies appeared while HIV antigen disappeared. Anti-CMV-IgM was clearly and anti-CMV-IgG questionably positive; IgM persisted further, while IgG remained definitely undetectable. We speculate that a particular HIV-induced imbalance of the immune system led to a generalized severe cytotoxic reaction to a simultaneous infection with CMV.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
7/13. Chronic infectious mononucleosis syndrome, pancytopenia, and polyclonal B-lymphoproliferation terminating in acute lymphoblastic leukemia.A 17-year-old previously healthy girl is reported who developed acute infectious mononucleosis followed by progressive ill health over 20 months, associated with pancytopenia and a polyclonal B-lymphoproliferation, terminating in acute lymphoblastic leukemia (ALL). Epstein-Barr virus (EBV) was recovered from the patient's nasopharyngeal secretions; serologic titers of antibodies to EBV-associated antigens were compatible with a chronic persistent EBV infection. plasma interferon levels were markedly elevated. EBV-specific cell-mediated immunity, as well as Natural killer (NK) cell activity were markedly deficient. Other studies of cell-mediated immunity revealed notable abnormalities, including abnormalities in T-cell subset ratios, and a serum blocker of autologous mitogen-induced lymphoproliferation. Humoral (plasma)-mediated, but not cell-mediated, suppression of hemopoiesis was demonstrated using in vitro erythroid and myeloid colony culture techniques. immunophenotyping of the patient's bone marrow cells preterminally was consistent with ALL. autopsy revealed pathologic changes of ALL in marrow and multiple organs. We conclude that our patient developed an EBV-driven lymphoproliferative disorder, with associated defective cell-mediated immunity and hemopoiesis. Ultimately, the patient's documented polyclonal lymphoproliferative state was superimposed by acute lymphoblastic leukemia.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/13. Fatal Epstein-Barr virus infection in a child with acute lymphoblastic leukemia in remission.A 9-year-old white boy developed a fatal primary Epstein-Barr virus (EBV) infection while receiving chemotherapy for acute lymphoblastic leukemia in remission. Histopathological findings at the height of the proliferative phase of the illness were compatible with a virally induced hemophagocytic syndrome. The infection spontaneously converted to complete aplasia of the bone marrow and lymph nodes. Serological studies disclosed that the patient had no antibodies to EBV prior to the infection, but during the acute phase he showed a spectrum and titers of antibodies to EBV-specific antigens characteristic of a current primary EBV infection. A lymph node biopsy obtained 5 weeks after onset revealed Epstein-Barr nuclear antigen in approximately 50% of the cells. The boy's condition deteriorated rapidly, with disseminated candidiasis resulting in cardiorespiratory failure and death. lymph nodes obtained at autopsy no longer contained Epstein-Barr nuclear antigen-positive cells.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
9/13. Cell-mediated immune-pancytopenia complicating primary sjogren's syndrome.A 64-year-old woman with mild bilateral parotid gland swelling and bilateral lower extremity purpura was admitted for evaluation of xerostomia and pancytopenia. The patient had an increased erythrocyte sedimentation rate, pancytopenia, and positive tests for antibodies to nuclear antigen, SS-A, and SS-B. Impaired cell-mediated immunity was also present. Bone marrow aspiration showed a hypoplastic marrow with an increased percentage of lymphocytes. A positive Schirmer's test and keratoconjunctivitis were also noted. A diagnosis of primary sjogren's syndrome was made by sialography and histological salivary gland findings. Therapy with prednisolone improved the pancytopenia. Addition of the patient's peripheral blood mononuclear cells to cultures of bone marrow mononuclear cells derived from a healthy volunteer dose dependently inhibited colony formation, including mixed hemopoietic colonies. On the other hand, addition of the patient's serum failed to inhibit colony formation by normal bone marrow mononuclear cells. These results suggested that one of the causes of pancytopenia in primary sjogren's syndrome might be mediated by the inhibition of mononuclear cells to the hemopoietic progenitors.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/13. adult respiratory distress syndrome with increased serum and bronchoalveolar lavage fluid levels of squamous cell carcinoma-related antigen.We report a case with adult respiratory distress syndrome (ARDS) associated with increased levels of squamous cell carcinoma-related antigen (SCC) in the serum and bronchoalveolar lavage fluid (BALF). ARDS was likely induced by ibuprofen, based on the presence of pancytopenia and a weakly positive drug lymphocyte stimulating test (DLST). High serum and BALF levels of interleukin (IL)-8, neutrophil elastase as well as SCC were detected. Corticosteroid therapy resulted in clinical improvement, resolution of pulmonary infiltrates on chest roentgenogram and normalization of serum and BALF levels of IL-8, neutrophil elastase and SCC.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
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