1/72. Congenital acute megakaryoblastic leukemia (M7) with chromosomal t(1;22)(p13;q13) translocation in a set of identical twins.Chromosomal translocations at t(1;22)(p13;q13) have been reported to occur in a number of infants with acute megakaryoblastic leukemia. A set of female twins with acute megakaryoblastic leukemia are reported with this unique translocation of 1p13 to 22q13. The twins presented at 2 months of age with fever and poor feeding and subsequently developed progressive hepatosplenomegaly. One twin died before treatment could be started; the other became septicemic 5 days after initiation of chemotherapy and eventually died.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/72. Long-term remission in an elderly patient with mantle cell leukemia treated with low-dose cyclophosphamide.We present an elderly patient with mantle cell leukemia who was successfully treated with low-dose cyclophosphamide (CY). A 76-year-old female was diagnosed as mantle cell leukemia based on abnormal lymphocytosis and splenomegaly without lymphadenopathy. She was orally treated with 50 mg of CY daily and had continuous remission over 4 years. Rearrangements of BCL1 and immunoglobulin heavy chain genes in the peripheral blood lymphocytes were detected at diagnosis, but not 1 or 4 years later. Further studies are required to confirm the role of low-dose CY therapy for patients with mantle cell leukemia and lymphoma.- - - - - - - - - - ranking = 1.1666666666667keywords = leukemia (Clic here for more details about this article) |
3/72. Intensive leukapheresis as initial therapy for chronic granulocytic leukemia.Intensive leukapheresis has been used as the initial treatment of chronic granulocytic leukemia (CGL) in six patients. The number of leukaphereses ranged from 3 in 7 days to 13 in 39 days (mean, 8 in 22 days). The procedures were well tolerated, and in all patients there was improvement in hematologic values, in most cases with considerable reduction in the peripheral leukocytosis and thrombocytosis and in the proportion of immature granulocytic cells in the circulation. splenomegaly decreased considerably in the four patients who had more than four leukaphereses. Symptoms of sweating, malaise, and pain due to splenomegaly were rapidly relieved. Problems due to hyperuricemia did not occur, but four patients required blood transfusions for correction of anemia. This method of initial treatment of CGL appears to give more rapid relief of symptoms than does conventional chemotherapy; it incurs no risk of hyperuricemia and lessens that associated with thrombocytosis. In addition, large quantities of granulocyte-rich plasma are made available for the treatment of infections in neutropenic patients. Intensive leukapheresis deserves more widespread evaluation as the initial treatment of CGL.- - - - - - - - - - ranking = 0.83333333333333keywords = leukemia (Clic here for more details about this article) |
4/72. Improvement of quality of life after splenectomy in an HTLV-I carrier with T-cell prolymphocytic leukemia.A 34-year-old woman of HTLV-I carrier with T-PLL, whose quality of life improved and survival was prolonged after splenectomy, is described. The patient had marked splenomegaly, generalized lymphadenopathy and marked proliferation of abnormal lymphocytes in the peripheral blood with an irregular nucleus, deeply basophilic cytoplasm and a single prominent nucleolus, which were positive for CD2, CD3, CD5, CD7, CD4 and CD8. Although the patient had serum antibody against HTLV-I, HTLV-I proviral dna integration was not detected. She was diagnosed as an HTLV-I carrier with T-PLL and received combination chemotherapy and 15.1 Gy splenic irradiation. However, the generalized lymphadenopathy and splenomegaly did not improve. The patient underwent splenectomy to palliate abdominal distension and hypersplenism. After the operation, her symptoms improved dramatically and within a week her hemoglobin concentration and platelet count normalized. She was discharged from hospital two weeks after the splenectomy, however 11 months later, she relapsed and despite treatment with chemotherapy and alpha-interferon, she died two months after the second admission. autopsy findings revealed that PLL cells had invaded the bone marrow, lymph nodes, liver, lungs, kidneys, uterus, ovaries and adrenal glands.- - - - - - - - - - ranking = 0.66666666666667keywords = leukemia (Clic here for more details about this article) |
5/72. Congenital monoblastic leukemia with 9;11 translocation in monozygotic twins : a case report.We report an autopsy case of congenital monoblastic leukemia that developed in monozygotic twins. The twin presented with progressive hepatosplenomegaly at 4 weeks after birth. One twin died of massive bleeding and hypovolemic shock before the treatment started. At autopsy, the liver was diffusely enlarged and showed a diffuse whitish discoloration except for the subcapsular and perivenular areas. Microscopic examination disclosed infiltration of histiocyte-like atypical cells along the sinusoids and portal areas of the liver. spleen, lymph nodes and choroid plexus were also infiltrated by the tumor cells. However, bone marrow involvement of the tumor was minimal although multifocal. On immunohistochemical staining, these atypical cells were reactive for CD68 (PGM-1) and lysozyme, suggesting that the tumor cells might have been derived from mono- histiocyte. Cytogenetic study revealed 9;11 translocation, which is frequently associated with acute monoblastic leukemia. To the best of our knowledge, this is the first report of congenital monoblastic leukemia of monozygotic twins in korea.- - - - - - - - - - ranking = 1.1666666666667keywords = leukemia (Clic here for more details about this article) |
6/72. Gamna-Gandy bodies of the spleen detected with MR imaging: a case report.We report a case of a female patient with portal hypertension due to liver cirrhosis. In this case, MR imaging revealed small siderotic nodules of the spleen, called Gamna-Gandy bodies. These lesions are found in patients with portal vein or splenic vein thrombosis, hemolytic anemia, leukemia, or lymphoma, patients receiving blood transfusions, acquired hemochromatosis, or paroxysmal nocturnal hemoglobinuria. There are only few reports in the literature about these siderotic nodules which are not very familiar. MR imaging seems to be the superior imaging method for detection of these lesions. It is important to consider Gamna-Gandy bodies in the differential diagnosis of portal hypertension and the other diseases mentioned above.- - - - - - - - - - ranking = 0.16666666666667keywords = leukemia (Clic here for more details about this article) |
7/72. Successful treatment with low-dose splenic irradiation for massive splenomegaly in an elderly patient with hairy-cell leukemia.The therapeutic approach to hairy-cell leukemia (HCL) is in some instances still debated. Although management with alpha-interferon and purine analogues is well established, there is an alternative role for therapeutic splenectomy in patients with massive splenomegaly who have failed to respond to systemic therapy. Most patients with HCL will not be suitable for treatment with splenectomy as their ages at diagnosis are high. Here, we report an elderly Japanese HCL patient whose refractory massive splenomegaly responded well to low-dose splenic irradiation.- - - - - - - - - - ranking = 0.83333333333333keywords = leukemia (Clic here for more details about this article) |
8/72. Nucleolated variant of mantle cell lymphoma with leukemic manifestations mimicking prolymphocytic leukemia.Chronic lymphoproliferative disorders sometimes can be difficult to classify. We report 4 cases characterized by large cells with distinct central nucleoli, reminiscent of prolymphocytic leukemia, but shown on further workup to represent mantle cell lymphoma. At initial examination, the patients had generalized lymphadenopathy, splenomegaly, and a leukemic blood picture. The peripheral blood showed many large cells with round to slightly irregular nuclei, single central nucleoli, and a fair amount of pale cytoplasm. The picture was not typical of prolymphocytic leukemia because of the presence of generalized lymphadenopathy and the large size of the circulating abnormal cells. Immunophenotypic study showed that the large lymphoid cells were CD5 CD23- mature B cells with overexpression of cyclin d1, and cytogenetic study demonstrated the translocation t(11;14)(q13;q32) in 3 patients. Lymph node biopsy confirmed a diagnosis of mantle cell lymphoma, pleomorphic variant, in all 4 patients. This study documents the existence of an unusual leukemic form of mantle cell lymphoma with prominent nucleoli; the clinicopathologic features that distinguish it from other chronic lymphoproliferative disorders are discussed.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
9/72. Hemophagocytosis and granulomas in the bone marrow of a child with down syndrome.Persistent fever with pancytopenia and hepatomegaly with negative blood cultures and no obvious focus of infection in a child with down syndrome should arouse a suspicion of leukemia. bone marrow examination and clot biopsy from one such patient revealed hemophagocytosis and granulomas, with serologic evidence of recent Epstein-Barr virus infection. Bone marrow granulomas are not a feature of Epstein-Barr infection. Later, bone marrow culture and repeat blood culture grew salmonella typhi. Thus, in a febrile child, when performing a bone marrow aspirate, a clot biopsy and culture for infectious etiology may be helpful, even when leukemia is strongly suspected.- - - - - - - - - - ranking = 0.33333333333333keywords = leukemia (Clic here for more details about this article) |
10/72. Lymphoid preponderance and the absence of basophilia and splenomegaly are frequent in m-bcr-positive chronic myelogenous leukemia.Chronic myelogenous leukemia (CML) with a minor bcr-abl transcript is a rare entity. We describe a 66-year-old female who was diagnosed with CML in the chronic phase. Molecular analysis of her philadelphia chromosome using the reverse transcriptase polymerase chain reaction and subsequent sequencing revealed a minor bcr-abl transcript. Monocytosis resembling chronic myelomonocytic leukemia was observed without splenomegaly and basophilia. Her clinical course was indolent and maintained the chronic phase of CML for nearly 3 years under hydroxyurea treatment. A review of the 23 cases of m-bcr CML including this case showed the presence of monocytosis and the absence of basophilia and splenomegaly in 55.0%, 55.0%, and 70.0% of patients, respectively. The absence of basophilia was a significant finding in patients without monocytosis ( P=0.01). Although the hematological features or clinical outcomes were variable in m-bcr CML cases, all three cases at the onset of the blastic phase showed lymphoid crisis, implying an increased lymphoid leukemogenicity of minor bcr-abl transcripts.- - - - - - - - - - ranking = 1.045882942656keywords = leukemia, myelogenous, myelogenous leukemia (Clic here for more details about this article) |
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