1/19. Bone marrow failure associated with human herpesvirus 8 infection after transplantation.BACKGROUND: Human herpesvirus 8 (HHV-8) infection has been linked to the development of Kaposi's sarcoma and to rare lymphoproliferative disorders. methods: We used molecular methods, serologic methods, in situ hybridization, and immunohistochemical analyses to study HHV-8 infection in association with nonmalignant illnesses in three patients after transplantation. RESULTS: Primary HHV-8 infections developed in two patients four months after each received a kidney from the same HHV-8-seropositive cadaveric donor. Seroconversion and viremia occurred coincidentally with disseminated Kaposi's sarcoma in one patient and with an acute syndrome of fever, splenomegaly, cytopenia, and marrow failure with plasmacytosis in the other patient. HHV-8 latent nuclear antigen was present in immature progenitor cells from the aplastic marrow of the latter patient. Identification of the highly variable K1 gene sequence of the HHV-8 genome in both the donor's peripheral-blood cells and the recipients' serum confirmed that transmission had occurred. HHV-8 viremia also occurred after autologous peripheral-blood stem-cell transplantation in an HHV-8-seropositive patient with non-Hodgkin's lymphoma. Reactivation of the infection was associated with the development of fever and marrow aplasia with plasmacytosis; there was no evidence of other infections. HHV-8 transcripts and latent nuclear antigen were expressed in the aplastic marrow but not in two normal marrow samples obtained before transplantation. CONCLUSIONS: Primary HHV-8 infection and reactivation of infection may be associated with nonneoplastic complications in immunosuppressed patients.- - - - - - - - - - ranking = 1keywords = blood cell (Clic here for more details about this article) |
2/19. Pearson marrow-pancreas syndrome with worsening cardiac function caused by pleiotropic rearrangement of mitochondrial dna.Pearson marrow-pancreas syndrome is a usually fatal disorder that involves the hematopoietic system, exocrine pancreas, liver, kidneys, and often presents clinically with failure to thrive. We report a 5-year-old patient who developed, in addition to the typical features of Pearson syndrome, worsening cardiac function, mainly affecting the left ventricle. The latter finding is particularly interesting because cardiac involvement has not yet been regarded as a major feature of Pearson syndrome. The diagnosis was proved by the finding of so far undescribed pleioplasmatic rearrangement of mitochondrial (mt)dna (loss of 5,630 bp, 70% deleted and duplicated mtDNA) in blood cells. Our report demonstrates that patients with Pearson syndrome may also have impaired cardiac function. Thus, Pearson syndrome should be considered in the differential diagnosis of patients with left ventricular dysfunction of unknown origin and other clinical findings suggestive of a mitochondrial disease.- - - - - - - - - - ranking = 1keywords = blood cell (Clic here for more details about this article) |
3/19. Bone marrow hypoplasia during brucella infection.pancytopenia, although mainly reported in adults, has also been described in children with brucellosis. However, bone marrow hypoplasia is a rare feature of the infection. An 11-year-old boy was admitted with fever, vomiting, and abdominal pain of 10 days' duration. On physical examination, pallor and high fever were detected in the absence of lymphadenopathy and hepatosplenomegaly. His hemoglobin was 8.6 g/dL, white blood cell count 1,100/mm(3), neutrophil count 500/mm(3), platelets 56,000/mm(3), and reticulocytes 0.1%. Hypocellular bone marrow was found by aspiration, and bone marrow biopsy revealed hypocellularity. The agglutination titer was greater than 1/640. trimethoprim/sulfamethoxazole was prescribed. His fever subsided and pancytopenia subsequently improved. pancytopenia associated with brucellosis is attributed to hypersplenism, hemophagocytosis, and granulomatous lesions of the bone marrow, which is usually hypercellular. Bone marrow hypoplasia is rarely reported and should be kept in mind in the etiology of aplastic anemia in a country where brucellosis is frequently encountered.- - - - - - - - - - ranking = 1.0350182288234keywords = blood cell, white (Clic here for more details about this article) |
4/19. Hematologic features and clinical course of an infant with Pearson syndrome caused by a novel deletion of mitochondrial dna.OBJECTIVE: Pearson bone marrow-pancreas syndrome (PS) is a rare, usually fatal mitochondrial disorder involving the hematopoietic system in early infancy. Due to the diversity of clinical symptoms, the diagnosis can be difficult. The authors describe a boy with severe hypoplastic anemia in whom extensive clinical, biochemical, and morphologic findings led to the diagnosis of PS, and molecular analysis revealed a novel deletion of mitochondrial dna from nucleotide position 10.371 to 14.607. methods: The patient is a 2-year-old boy who presented at age 5 months with hypoplastic macrocytic anemia. His first months of life and the family history were uneventful. Extensive pretransfusion evaluations did not reveal a metabolic, infectious, or hematologic-neoplastic etiology, and he had no evidence of exocrine pancreatic insufficiency. However, a second bone marrow aspirate at age 7 months showed a reduced cell number, vacuolated erythroblasts and myeloblasts, and ringed sideroblasts, so PS was suspected. RESULTS: Additional molecular analysis from the boy's blood leukocytes revealed a deletion of mitochondrial dna from nucleotide position 10.371 to 14.607, which was absent in his mother's blood cells, consistent with a sporadic mutation as commonly seen in PS. The muscle histology and the respiratory chain enzymes were normal. CONCLUSIONS: Mitochondriopathies should be considered in children with persistent non-neuromuscular symptoms such as unexplained refractory anemia. Due to the often-fatal course of PS, the rapid detection of mitochondrial dna deletions is imperative for diagnosis and family counseling.- - - - - - - - - - ranking = 1keywords = blood cell (Clic here for more details about this article) |
5/19. Purine nucleoside phosphorylase deficiency associated with a dysplastic marrow morphology.Purine nucleoside phosphorylase (PNP) deficiency is an autosomal recessive metabolic disorder characterized by severe combined immunodeficiency and by complex neurologic symptomatology including ataxia, developmental delay, and spasticity. Herein we report severe marrow dysplasia in a patient with PNP deficiency. Drug-related marrow dysfunction was unlikely, and marrow virological studies were negative. A preleukemic myelodysplastic syndrome was also unlikely due to normal marrow CD34 cells, colony growth in clonogenic assay of marrow mononuclear cells, apoptosis rate, and Fas expression on marrow nucleated cells, as well as morphologic improvement of the marrow dysplasia after normal red blood cell transfusion. The patient's marrow stroma showed hypersensitivity to irradiation and undetectable PNP enzyme activity similar to peripheral lymphocytes. This is the first report of PNP deficiency associated with increased lymphocyte and marrow stromal sensitivity to irradiation. We conclude that marrows from patients with PNP deficiency might have hypersensitivity to irradiation and can develop dysplastic morphology, caused either directly or indirectly by the inherited enzymatic defect.- - - - - - - - - - ranking = 1keywords = blood cell (Clic here for more details about this article) |
6/19. Bone marrow necrosis in a patient with acute promyelocytic leukemia during re-induction therapy with arsenic trioxide.arsenic trioxide (As2O3) therapy at a daily dose of 0.15 mg/kg was given to a 60-yr-old Japanese male with refractory acute promyelocytic leukemia. White blood cell (WBC) of 6.6 x 10(3)/microl increased to 134 x 10(3)/microl following the administration of As2O3. Daily hydroxyurea (HU), and 6-mercaptopurine (6-MP) were added on days 7 and 19, respectively. Both HU and 6-MP were discontinued on day 28, when WBC declined to 54.0 x 10(3)/microl. He developed unexplained fever and profound cytopenia requiring multiple blood products transfusions. bone marrow examination on day 42 revealed massive necrosis. pharmacokinetics confirmed a mean maximum plasma arsenic concentration (Cpmax) and a half-life time (t1/2) of 6.9 microm and 3.2 h, respectively, in the therapeutic range. This is the first case of bone marrow necrosis after standard-dose As2O3 therapy.- - - - - - - - - - ranking = 1keywords = blood cell (Clic here for more details about this article) |
7/19. lymphoma in a patient with systemic lupus erythematosus.BACKGROUND: A 40-year-old woman with a 10-year history of systemic lupus erythematosus (SLE) presented with fever, lymphadenopathy and fatigue. Before that time, her SLE symptoms had been controlled with hydroxychloroquine, NSAIDs, and an occasional short course of moderate-dose prednisone. Two months before presentation, she experienced fevers ranging from 38.3 to 39.7 degrees C, but she had no specific symptoms that suggested local infection. INVESTIGATIONS: physical examination, multiple blood cultures, and laboratory investigations that included the following tests: hemoglobin concentration; erythrocyte sedimentation rate; c-reactive protein level; serum lactate dehydrogenase level; aspartate aminotransferase level; alanine aminotransferase level; serum complement c3 and C4 levels; white-blood-cell count; platelet count; urinalysis; serum creatinine level; CT of the chest and abdomen; bone-marrow biopsy; serum electrophoresis; and tests for Epstein-Barr virus, cytomegalovirus, hepatitis b virus, hepatitis c virus, hiv-1, antinuclear antibodies, antibodies to Smith antigen, antibodies to double-stranded dna, and antibodies to Ro and La. diagnosis: Stage IVB diffuse large B-cell lymphoma with marrow and liver involvement concurrent with SLE. MANAGEMENT: The patient promptly underwent chemotherapy, receiving three courses of 3 mg/m(2) vindesine on day 1, 1,500 mg/m(2) cyclophosphamide and 80 mg/m(2) doxorubicin on day 2, and 50 mg/m(2) prednisolone on days 1-5.- - - - - - - - - - ranking = 0.035018228823353keywords = white (Clic here for more details about this article) |
8/19. Reversible bone marrow hypoplasia induced by alcohol.A 51-year-old man developed severe pancytopenia with bone marrow hypoplasia after consuming excessive amounts of alcohol for many years. After avoiding alcohol and eating normally for about 10 days, his blood cell counts promptly recovered to near-normal levels. Hematologic remission had lasted for 7 months until he began to drink alcohol excessively again, leading to a relapse of pancytopenia with marrow hypoplasia. His blood cell counts attained near-normal levels again after abstaining from alcohol for about a month. He developed thrombocytopenia following moderate ingestion of alcohol 7 months later, but he never developed severe pancytopenia after limiting alcohol ingestion. When bone marrow mononuclear cells from the patient and normal volunteers were cultured in the presence of ethanol, CFU-GM-derived colony formation of the patient was inhibited by much lower concentrations of ethanol than that of normal volunteers. The clinical course of the patient and the greater sensitivity of the patient's CFU-GM to ethanol toxicities indicate that massive intake of alcohol was responsible for bone marrow hypoplasia observed in this patient.- - - - - - - - - - ranking = 2keywords = blood cell (Clic here for more details about this article) |
9/19. Kaposi's sarcoma with visceral involvement in a young heterosexual male without evidence of the acquired immune deficiency syndrome.A case of disseminated Kaposi's sarcoma (KS) in a 21-yr-old white heterosexual male with cryptogenic cirrhosis and no serological or immunological evidence of acquired immune deficiency syndrome (AIDS) is reported. The patient died 2 wk after diagnosis. Postmortem examination showed involvement of lymph nodes, liver, spleen, gastrointestinal tract, and bone marrow. This case demonstrates that Kaposi's sarcoma can occur in a young heterosexual male with normal immune function and in the absence of HIV infection.- - - - - - - - - - ranking = 0.035018228823353keywords = white (Clic here for more details about this article) |
10/19. Marrow myxedema. Gelatinous transformation of marrow ground substance in a patient with severe hypothyroidism.Replacement of marrow ground substance by hyaluronic acid-rich mucopolysaccharides (gelatinous transformation) has been previously reported to occur in severely malnourished patients. A patient with severe anemia and hypothyroidism without malnutrition was found to have gelatinous transformation of the marrow. This process is similar histologically to dermal myxedema, and the findings in this patient suggest questions for further study involving possible roles for thyroid-stimulating hormone in the development of marrow and visceral myxedema and the alterations in the normal partitioning process between serum and red blood cell low-density lipoproteins that produce acanthocytes in blood smears from patients with hypothyroidism.- - - - - - - - - - ranking = 1keywords = blood cell (Clic here for more details about this article) |
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