Gene/Protein Disease Symptom Drug Enzyme Compound
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Chronic neutrophilic leukemia is a rare, infrequently recognized, myeloproliferative disorder. It usually manifests as a leukemoid reaction, with mostly mature granulocytes in the peripheral blood, with rare to occasional immature forms, and sometimes with normoblasts. The clinical manifestations also include hepatosplenomegaly, elevated leukocytic alkaline phosphatase, elevated serum vitamin B12 and serum vitamin B12 binder ("R" fraction), and elevated serum uric acid. Distinction from a leukemegaly, the absence of sepsis, usually normal erythrocytic sedimentation, and the absence of fever. Leukemoid reactions may be associated with elevated serum vitamin B12 and uric acid, but the levels are usually lower than those found in chronic neutrophilic leukemia. Many patients have gouty symptoms, especially after treatment with Busulfan, and many have an unexplained hemorrhagic tendency, making major operations a risk. The authors add two cases to the 11 previously described.
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PMID:Chronic neutrophilic leukemia. Report of two cases and review of the literature. 28 88

We have analyzed the indications and results of shunt operation versus orthotopic liver transplantation (OLT) in 22 patients with Budd-Chiari syndrome (BCS). The underlying cause of the syndrome was similar between the two groups and was related to myeloproliferative disorders or the use of birth control pills in 18 of 22 patients. The results of biopsies of the liver showed centrilobular congestion and necrosis in all candidates who underwent shunting and the presence of fibrosis and cirrhosis in the OLT candidates. The indications for shunts included symptoms related to portal hypertension only and well-preserved synthetic hepatic function. Ten patients were treated with 12 shunt procedures, including mesoatrial (eight patients) and side to side portacaval shunt (four patients). Significant complications after shunt procedure included fulminant (one of ten patients) and progressive (one of ten patients) hepatic failure requiring urgent OLT; one death occurred because of pulmonary sepsis. Indications for OLT were signs of end stage liver expressed by severe portal hypertension and variceal bleeding (four of 14 patients), progressive encephalopathy (seven of 14 patients) and poor synthetic function (bilirubin greater than 3 milligrams per deciliter in eight of 14 patients and albumin less than 3.0 grams per liter, or both, in ten of 14 patients). Fourteen patients were treated with 16 OLT, three patients had retransplantation for primary nonfunction graft (two of 14 patients) or chronic rejection (one of 14 patients). There were two early deaths in the group. With a follow-up period between two months to five years, 12 of 14 patients undergoing OLT are alive, fully functional and have normal liver function tests. Seven of ten patients who had shunts are alive, six are able to maintain normal activity and one has progressive end stage hepatic disease and is not a candidate for OLT. However, the hepatic function continues progressively to be abnormal. Various options are available for the treatment of the syndrome. Portosystemic decompression is effective and should be considered at the early stage of the disease, prior to the development of significant hepatic failure. However, few of the patients will continue to have slow, but progressive hepatic failure and may require OLT. The only effective treatment for end stage hepatic disease secondary to the BCS is OLT.
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PMID:Portosystemic shunt versus orthotopic liver transplantation for the Budd-Chiari syndrome. 159 20

A 52-year-old Japanese man manifested various clinical signs and symptoms such as vomiting, high fever, dyspnea, cough, sweating, palpitation, eosinophilic leukocytosis and hepatosplenomegaly. These histamine-related clinical manifestations showed a dramatic response to steroid therapy. After 10 months of hospitalization, he suddenly succumbed to candidal septicemia at the end of the third cycle of steroid therapy. Autopsy revealed neoplastic proliferation of immature basophils in various internal organs without involvement of the skin. The neoplastic cells, positive immunohistochemically for leukocyte common antigen, possessed lobulated nuclei and weakly metachromatic cytoplasmic granules, predominantly of the basophil type, which exhibited weak naphthol ASD-chloroacetate esterase activity. Mast cell-type granules were also observed ultrastructurally. The neoplastic infiltration was associated with fibrosis in the liver, spleen and bone marrow and with extramedullary hematopoiesis in the liver, spleen, lymph nodes and perihypophyseal tissue. The bone marrow showed uneven and multifocal involvement. Despite the lack of leukemic manifestations and the results of chromosomal analysis, the most suitable diagnosis was aleukemic basophilic leukemia within the category of chronic myeloproliferative disorder. Kinship of this neoplasia to systemic mastocytosis is discussed.
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PMID:An unusual form of chronic myeloproliferative disorder. Aleukemic basophilic leukemia. 203 58

Dental disorders have been recognized as major sources of infection in patients with hematologic malignancies (HM). Management of severe dental infections usually includes dental extractions (DE), but the safety of extractions in patients with HM who are at risk for bleeding, sepsis, and poor wound healing has not been well established. In conjunction with an aggressive program of dental care, 142 DE were performed in 26 patients with acute leukemia, myelodysplastic syndromes, and myeloproliferative disorders. Granulocytopenia (less than 1,000 granulocytes/microL) was present during or within ten days following surgery in 14 patients. In these 14 patients (101 DE), the mean granulocyte count was less than 450/microL, with a median duration of granulocytopenia following surgery of 32 days (range, four to 169 days). Thrombocytopenia (less than 100,000 platelets/microL) occurred during or within two days following surgery in 13 patients (80 DE), with a mean platelet count of 63,500/microL. Transfusions were given for platelet counts less than 50,000/microL. All DE were performed without significant complications. Bleeding was minor to moderate and easily controlled with local measures; no patient required transfusion due to hemorrhage. Average maximum temperature 24 hours after DE was 37.7 degrees C. No episodes of bacteremia were documented within ten days of DE. Minor delay in wound healing was observed in two patients. We conclude that DE can be safely performed in patients with HM in combination with aggressive supportive care.
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PMID:The safety of dental extractions in patients with hematologic malignancies. 252 58

A 16 year review of 391 splenectomies performed at New England Medical Center was done to evaluate the morbidity and mortality of patients with drained splenic weights greater than 1,000 grams. Thirty-six met the criteria for study. Twenty men and 16 women with an average age of 55.4 years were identified. Myeloproliferative disorders were the most predominant cause of massive splenomegaly. Pancytopenia and hemolytic complications of the disease processes were the most acute indications for operations. The average time between diagnosis and operative intervention was 42 months. An average of 10 units of blood products were required to correct preoperative coagulopathy. Eleven of 36 patients had postoperative complications. Eight of 21 with drains and an equal number of patients with preliminary splenic arterial ligation had complications. Eight-one per cent of all complications were infection related. Complication increased the length of stay 11 days. The 30 day mortality rate was 11.1 per cent. Sepsis was the major cause of mortality. Closed drainage system provided no demonstrable benefit nor appeared to be the cause of sepsis. No episodes of pulmonary embolic phenomenon or peripheral venous thrombosis were demonstrated. Elective splenectomy in patients with smaller spleens was performed without operative mortality and with 3 per cent morbidity rate. For patients with massive splenomegaly, the average survival time was 28.5 months. The majority of these patients died from complications of the disease. Preoperative coagulopathy, failure to demonstrate a hematologic response to splenectomy and reoperation were clear predictors for decreased long term survival periods.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Massive splenomegaly. 291 90

Twenty patients (age range, 4 to 48 years; median age, 36 years) with de novo or drug-induced myelodysplastic syndromes or myeloproliferative disorders were treated with myeloablative immunosuppressive therapy followed by bone marrow transplantation (BMT). Four preparative regimens were used; three regimens consisted of combined total body irradiation (TBI) and chemotherapy and one of combination chemotherapy only. One patient received marrow from his identical twin brother, whereas the other 19 patients were grafted with marrow from histocompatible siblings. In 19 patients the abnormal clone was at least temporarily ablated, while in one patient the congenital myelodysplasia persisted. Eight patients are alive and well for +108 to +3,359 days post-transplantation. Nine patients died of transplant-related complications (six of interstitial pneumonia, two of gastrointestinal bleeding, and one of fungal sepsis) and three patients died with persisting or recurring disease. One patient with a late recurrence has undergone a second successful bone marrow transplant procedure. Outcome of BMT was not related to French-American-British (FAB) type, marrow fibrosis, cytogenetic abnormalities, or preparation regimen. Marrow transplantation as a means of providing long-term disease-free survival and possible cure should be considered in patients if a suitable donor is available.
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PMID:Bone marrow transplantation for myelodysplastic and myeloproliferative syndromes. 331 15

Classification of platelet disorders has been based on the platelet count. Addition of a second variable, mean platelet volume (MPV), to the routine blood count allows classification of patients into 9 categories: high, low, or normal MPV, and high, low or normal platelet count. We studied 1,244 adult inpatients. 1,134 had both platelet values normal. 11 patients had high MPV and low platelet count: all had hyperdestructive causes. 15 patients had high MPV and normal platelet count: 12 had heterozygous thalassemia, and three had iron deficiency. Seven patients had high MPV and high platelet count: causes included myeloproliferative disorders, inflammation, iron deficiency, and splenectomy, 25 patients had high platelet counts and normal MPV: the causes were inflammation, infection, sickle cell anemia, iron deficiency, or chronic myelogenous leukemia. 52 patients had an MPV that was inappropriately low for the platelet count (high, normal, or low). All had sepsis, splenomegaly, aplastic anemia, chronic renal failure, or a disease being treated with myelosuppressive drugs. High MPV thus appears correlated with myeloproliferative disease or thalassemia; and low MPV, with cytotoxic drugs or marrow hypoplasia. Addition of MPV to the platelet count allows subtler disorders to be detected (when the platelet count is normal), and allows distinction of the cause of thrombocytopenia.
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PMID:Use of mean platelet volume improves detection of platelet disorders. 407 87

A 66-year-old female patient complained of loss of body weight and fatigue. The clinical examination revealed a thrombocytosis with a maximum count of 3.200 . 10(9) platelets and a leukocytosis with maximally 25 . 10(9) white cells in the peripheral blood. The bone marrow showed a large increase of megakaryocytes. Under the diagnosis of megakaryocytic myelosis a chemotherapy with 186 mg busulfan was performed. In the course of this treatment the clinical picture of a sepsis occurred which could not be controlled by antibiotics. The patient died four months after her admission to the clinic. The essential findings in autopsy were a caseous tuberculosis of the lymph nodes with haematogenic generalization which appeared as a septic tuberculosa gravissima ("typhobacillosis" Landouzy). The bone marrow was atrophic. Spleen liver and lymph nodes were without evidence for a myeloproliferative disorder. Thus, the initial diagnosis had to be changed to a megakaryocytic pseudomyelosis with massive thrombocytosis as a reaction to the tuberculous infection. The differential diagnosis of megakaryocytic myelosis, other disorders of the myeloproliferative syndrome, and the reactive thrombocytosis are discussed.
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PMID:[Megakaryocytic pseudomyelosis with severe thrombocytosis]. 617 Nov 9

Myelofibrosis is a proliferative response of the bone marrow fibroblasts. Myelofibrosis can be classified as primary or secondary depending on the underlying etiology. Primary myelofibrosis is a myeloproliferative disorder in humans in which there is a clonal proliferation of a pluripotent stem cell. Hemopathology includes finding nucleated red blood cells and immature granulocytes in the circulation, extramedullary hematopoiesis, and myelofibrosis. The proliferation of the bone marrow fibroblasts is not clonal in origin. To the best of this author's knowledge, this type of myelofibrosis has not been reported to occur naturally in the dog. Secondary myelofibrosis has been reported in the dog associated with neoplastic conditions, irradiation, congenital hemolytic anemias, and a variety of unknown etiologies. It has been shown in some cases of myelofibrosis that there is often concurrent bone marrow necrosis. Bone marrow necrosis has been documented in dogs with Ehrlichiosis and septicemia, and associated with drug treatment, including estrogens and cephalosporins. It is though that this necrosis is due to the destruction of the bone marrow microvasculature and/or hematopoietic elements. Release of growth factors by inflammatory cells may lead to the subsequent fibroblast proliferation. Several cases of secondary myelofibrosis in female laboratory beagles have been recently observed. These dogs present with a severe nonregenerative anemia and often a mild neutropenia with varying degrees of myelofibrosis in the bone marrow. Some animals have had concurrent bone marrow necrosis. At this time, the exact etiology is unknown.
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PMID:A review of myelofibrosis in dogs. 821 Sep 38

Two cases of acute leukemia with a t (6;9) (p23;34) chromosome abnormality are reported. The first case was a 34-year-old female who was hospitalized in October 1989. A diagnosis of FAB-M1 was made. Chromosomal analysis of the bone marrow cells showed a 46, XX, t (6;9) (p23;q34). Complete remission was achieved after two courses of BHAC-DMP therapy. In September 1991, at the time of relapse, chromosomal analysis revealed two abnormal clones consisting of a 46, XX, t (6;9) (p23;q34), -12, -17, +der (12) t (12;17) (p11.2;q11.2) with a residual normal clone. She died in February 1992. The second case was a 42-year-old male who was hospitalized in January 1990. He was diagnosed as having RAEB. Chromosomal analysis of the bone marrow cells showed 46, XY, t (6;9) (p23;q34). Three months later, the disease progressed to acute leukemia accompanied by leg ulceration with leukemic cell infiltration. Small-dose ara-C therapy was given, but with no effect. After two subsequent courses of therapy with low-dose etoposide, complete remission was achieved. Four months later, relapse occurred, and the patient died of sepsis in February 1991. In the literature, 31 cases of myeloproliferative disorders with t (6;9) have been reported.
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PMID:[Two cases of acute leukemia with t(6;9) (p23;q34)]. 845 Jun 8


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