Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 66-year-old man was presented with thrombocytosis in February, 1988. Laboratory examinations on admission revealed a white blood cell count of 17,700/microliters and a platelet count of 274.4 x 10(4)/microliters. Bone marrow aspirates showed an increase of megakaryocytes (1,294/microliters). There was no fibrosis or Ph1 chromosome. He was diagnosed as having essential thrombocythemia and was treated with thrombopheresis, carboquone and ranimustine (MCNU). Subsequently his platelet count was well controlled approximately for three years. He was readmitted because of pyrexia and left hypochondralgia in February 1991. Physical examination revealed hepatosplenomegaly. Peripheral blood revealed leukoerythroblastosis associated with the occurrence of tear drop cells. Bone marrow aspiration resulted in a dry tap and the biopsy specimen showed reticulin fibrosis. This is a fairly rare case of essential thrombocythemia that transformed to myelofibrosis.
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PMID:[Essential thrombocythemia that transformed to myelofibrosis after three years]. 160 18

We describe a case of multiple myeloma associated with myelofibrosis. This patient had hepatosplenomegaly, moderate anemia with anisocytosis and nucleated red blood cells, and Bence-Jones protein (kappa) in the urine. A bone marrow biopsy showed extensive marrow fibrosis and proliferation of numerous immature plasma cells containing kappa light chain in the cytoplasm. Melphalan-prednisolone therapy not only facilitated the disappearance of the immature plasma cells but also resulted in an improvement of myelofibrosis in the bone marrow. The immature plasma cell proliferation and marrow fibrosis in the bone marrow were seen again after interruption of chemotherapy. Therefore, this myelofibrosis may be secondary to the coexistent multiple myeloma.
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PMID:Multiple myeloma with coexistent myelofibrosis: improvement of myelofibrosis following recovery from multiple myeloma after treatment with melphalan and prednisolone. 180 51

The case histories of two patients with benign systemic mastocytosis with skin involvement are presented. The first patient had urticaria pigmentosa diagnosed at the age of 2 months, and developed systemic disease within two years. The second presented urticaria pigmentosa at the age of 22 years while benign systemic mastocytosis was diagnosed 30 years later. The clinical findings in both cases were: skin involvement, hepatosplenomegaly and abdominal pain. The second patient had myelofibrosis. There was a favorable response to H1 and H2 histamine antagonist and ketotifen.
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PMID:[Benign systemic mastocytosis: report of 2 cases]. 184 75

A 17 year old male was admitted because of pancytopenia. Bone marrow aspiration revealed myelodysplasia, no increase of blast cells and excessive expansion of megakaryocytic lineage. Although mild increase of bone marrow reticulin fiber was observed, no hepatosplenomegaly was recognized. Therefore he was diagnosed as refractory anemia (RA) or MDS with myelofibrosis and treated with low dose Ara-C regimen. Remission was achieved in June 1987, but the relapse occurred in Oct. 1987. His bone marrow at the relapse showed more remarkable dysplastic change than before. Sequential bone marrow examinations thereafter, revealed an increase of megakaryocytic lineage, especially immature dysplastic megakaryocytes, leading to the appearance of the abnormal megakaryoblasts (detected with anti GP IIb/IIIa antibody) as well as uncharacterized blast cells in his terminal stage. Transformation from MDS to megakaryocytic leukemia was strongly suggested. He died of severe pneumonia in March 1989. The invasion of abnormal immature megakaryocytic cells including megakaryoblasts was observed in liver, spleen and lymph nodes at autopsy. There are several reports on cases having a common hematological features such as 1) pancytopenia in peripheral blood, 2) myelodysplasia, 3) excessive growth of megakaryocytic lineage, 4) myelofibrosis without hepatosplenomegaly, although other clinical features were different. We propose all these cases should be reviewed at the point of MDS mainly involved in megakaryocytic lineage.
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PMID:[Myelodysplasia predominantly involving in megakaryocytic lineage successfully treated with low-dose Ara-C]. 194 32

From 1982 to 1985, four cases of primary myelofibrosis were diagnosed in our department. Three were boys and one was a girl. Their ages ranged from 7 months to 15 years. The diagnosis was made based on anemia, leukoerythroblastic change and presence of giant platelets in the peripheral blood, and a bone marrow biopsy showing myelofibrosis. Most of them had anemia, fever, and hepatosplenomegaly on admission. The anemia was severe and refractory to repeated transfusions and steroid therapy in 3 out of the 4 cases. Splenectomy was performed in 1 case, but without satisfactory results. The clinical course and blood pictures in one case resembled leukemia of megakaryocyte lineage (M7), but results of marker studies of the blast cells ruled out the possibility of M7. Three of them underwent leukemic transformation within 2 years and died soon after. The other one died of sepsis 2 weeks after diagnosis. Myelofibrosis in childhood occurs rarely, however, when it does, it always runs a rapid and fatal course.
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PMID:Primary myelofibrosis in children: report of 4 cases. 198 Dec 37

A case of bone marrow myelofibrosis with extramedullary hematopoiesis in the prostate gland is described. This 75-year-old man had an 8-year history of myelofibrosis, massive hepatosplenomegaly, and progressive anemia and a 5-year history of bladder outlet obstruction symptomatology. Transurethral resection of prostate was performed, and light microscopic examination of the prostatic chips revealed a diffuse distribution of atypical megakaryocytes, immature granulocytes, and normoblasts in the prostatic stroma. The prostatic glandular epithelium was uninvolved. Immunostaining for hemoglobin in erythroid precursors and a chloroacetate esterase stain for granulocytic precursors were useful in confirming the diagnosis and in defining the extent of prostatic involvement. Extramedullary hematopoiesis is a rare process in the prostate gland, but it should be included in the differential diagnosis of cellular prostatic stromal lesions, particularly when atypical giant cells are observed.
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PMID:Extramedullary hematopoiesis in the prostate. 203 42

A 74-year-old man was admitted to the National Defense Medical College Hospital because of purpura on the extremities. The blood hemoglobin level was 11.5 g/dl, the white blood cell count 7. 3 x 10(9)/l with 3% blastic cells, and the platelet count 4.0 x 10(9)/l. There was leukoerythroblastosis with only mild teardrop appearance of erythrocytes. The blastic cells consisted of two types, megakaryoblast-like cells and myeloblast-like cells. There was a very mild hepatosplenomegaly, but no lymphadenopathies. The bone marrow aspirations yielded dry taps. The marrow biopsy specimen revealed myelofibrosis associated with trilineage hyperplasia, but no apparent infiltration of leukemic cells. Platelet-associated IgG (PAIgG) had a high titer and the response to platelet transfusions was poor. On prednisolone and colchicine, the blastic cells in the peripheral blood disappeared and the platelet count increased. He died of pneumonia. The autopsy showed myelofibrosis with hyperplasia of myeloid and erythroid lineage in the bone marrow. Megakaryocytes were relatively few and there was no infiltration of leukemic cells.
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PMID:[Myelofibrosis associated with immunological abnormalities]. 221 80

Idiopathic myelofibrosis is a disease of unknown cause characterized by systemic marrow fibrosis and extramedullary hematopoiesis. We report here a patient of myelofibrosis treated successfully by busulfan pulse therapy which was reported first by chang et al in 1988. The patient was a 62-year-old woman who was referred to us for anemia and hepatosplenomegaly in August 1984. Further examination established a diagnosis of idiopathic myelofibrosis. During the subsequent 4-year follow-up period without specific treatment in our outpatient clinic, there occurred gradual progression of anemia and hepatosplenomegaly with the spleen extending beyond the level of the umbilicus. In September 1988, she was initiated on 4-day pulse therapy of busulfan with a daily dose of 12 mg, which was repeated 10 times until July 1989. This was followed by marked improvement of anemia and hepatosplenomegaly. Post-treatment iliac marrow biopsy showed some reduction of reticulin fibers with increased hematopoietic elements as compared to pretreatment iliac marrow biopsy. The busulfan pulse therapy, therefore, appears to be a treatment of choice in idiopathic myelofibrosis.
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PMID:[Successful treatment of primary myelofibrosis by busulfan pulse therapy: report of a case]. 224 32

We report a case of acute myelofibrosis (AMF) developing into acute myelomegakaryoblastic leukemia. A 33-year-old woman was admitted to our hospital because of fever and chest pain. On physical examination, hepatosplenomegaly was not noticed. Pancytopenia and a small number of blast cells were observed in the peripheral blood. Poikilocytosis was not detected. Bone marrow examination revealed dry tap on aspiration, and moderate increase in reticulin fiber on biopsy. The diagnosis of AMF was made. Eight months later, blast cells markedly increased. Surface marker was investigated and MCS-2 (CD13), C17 (CDw41) and P2 (CDw41) were found to be positive. Electron microscopic examination revealed that blast cells were composed of PPO-positive cells and MPO-positive cells. Based on these findings, it was considered that the patient developed acute myelomegakaryoblastic leukemia. Recently AMF is thought to be a state to have the ability to develop into various types of acute leukemia. Adequate therapy may be required before the development of leukemia.
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PMID:[Acute myelofibrosis terminating in acute myelomegakaryoblastic leukemia]. 259 46

A case of peripheral T-cell lymphoma presenting with secondary myelofibrosis and meningeal involvement is described. A 65-year-old female was admitted because of remarkable weight loss and pancytopenia. On admission, she was confused and showed tiny cervical lymph nodes but no hepatosplenomegaly. Bone marrow aspiration resulted in dry tap and its biopsy showed remarkable myelofibrosis with marked decrease of hematopoiesis and increase of lymphoid cells. Lymph node biopsy revealed diffuse medium sized cell lymphoma, which was diagnosed as CD3+4+8-peripheral T-cell lymphoma with immunohistochemistry (anti-HTLV-1 antibody negative). The lymphoid cells of bone marrow expressed the markers of T-cell lineage (LCA+ UCHL1+ MT1+ L26- MB1-). The cerebrospinal fluid examination revealed many lymphoma cells. She was treated with CHOP regimen and intrathecal injection of MTX. After three months, bone marrow biopsy showed recovery of hematopoiesis and disappearance of lymphoma cells and reticulin fibers. Immunohistochemical analysis of bone marrow specimen was useful for the diagnosis of atypical myelofibrosis.
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PMID:[Peripheral T-cell lymphoma initially presenting as secondary myelofibrosis]. 269 63


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