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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An unusual syndrome of hepatosplenomegaly and fever followed by rapid deterioration and death has been described in 38 children from 21 families. Pancytopenia, liver dysfunction, and bleeding developed prior to death from hemorrhage, sepsis, or lymphocytic meningitis. This report reviews the literature and adds a set of twins to the reported cases.
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PMID:Familial erythrophagocytic lymphohistocytosis. Report of two cases and clinicopathologic review. 77 51

In two patients with extensive marrow necrosis, the diagnosis of marrow necrosis was established by morphologic and radioisotopic studies, and the extent of involvement was accurately assessed by marrow scanning with technetium Tc 99m sulfur colloid while the patients were still alive. The literature on marrow necrosis was briefly reviewed and the clinical features of this condition were characterized. It was found that patients with this condition often have malignancies, underlying marrow disorders, sepsis, bone pain, and pancytopenia. Their marrow is often difficult to aspirate and they may require frequent transfusions to maintain a stable hemoglobin level. Radioisotopic studies are useful in the diagnosis and assessment of extent of involvement of this condition. They should be used in patients with clinical findings suggestive of marrow necrosis.
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PMID:Bone marrow necrosis. Diagnosis and assessment of extent of involvement by radioisotope studies. 87 25

Thirty-one patients with chronic lymphocytic leukemia were treated with mediastinal radiation. In none of the patients was complete remission achieved; either partial remission or clinical improvement was achieved in 52 per cent, but the duration of response was short. The response rate was 77 per cent for the patients receiving a total radiation dose greater than 3,000 rads and 45 per cent for those receiving less than 3,000 rads. Severe life-threatening toxicity was noted in 11 patients and seven of these patients died; two patients died with progressive disease. Severe toxicity was manifested by one or more of the following: bone marrow aplasia, pancytopenia, gram-negative sepsis, generalized herpes zoster and severe esophagitis. Neither the total dose of radiation nor the dose per week correlated withe the severity of reaction or death.
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PMID:Mediastinal irradiation for chronic lymphocytic leukemia. 100 73

Report of a T-cell rich B-cell lymphoma (TCRBCL) in a 43 years old man with an associated haemophagocytic syndrome (HS). At presentation the haemophagocytic cells involved the same organs as the lymphoma, i.e. spleen, liver, abdominal lymph nodes and bone marrow. As supportive measure to alleviate chemotherapy-induced granulocytopenia the cytokine granulocyte-macrophage colony-stimulating factor (GM-CSF) was given. After an initial improvement of the blood granulocyte count pancytopenia developed again, resulting in fatal sepsis. Autopsy demonstrated massive proliferation of macrophages in the bone marrow with haemophagocytosis as morphological correlation to the pancytopenia. The observation that exogenous GM-CSF enhanced the preexistent HS primarily reactive to the TCRBCL raises the question if endogenous GM-CSF may play a role in triggering a HS. The observed association of TCRBCL and HS has not been reported so far.
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PMID:[T-cell rich B-cell lymphoma associated with hemophagocytic syndrome]. 128 41

Seven children with Gaucher's disease who underwent partial splenectomy were followed for 7 to 8 years. None of the children had systemic sepsis or symptoms related to liver enlargement. Bone crisis occurred in only two children who had experienced bone crisis prior to partial splenectomy. This contrasts with the development of bone crises in five of six children who underwent total splenectomy at a similar age and who had previously been free of bone symptoms. Partial splenectomy should be regarded as a temporary solution in the treatment of hypersplenism and the mechanical compression related to the huge spleen. In 71% of the patients, massive enlargement and a severe to moderate degree of pancytopenia occurred again after 3 to 8 years; total splenectomy was required in three patients.
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PMID:Long-term follow-up of partial splenectomy in Gaucher's disease. 141 42

A total of 56 patients were diagnosed as primary myelodysplastic syndrome (MDS) at Chang Gung Memorial Hospital, Kaohsiung from April 1986 to December 1991. The median age was 65 years with an equal sex ratio. All patients presented with anemia and 52% with pancytopenia. The overall median survival for the entire group was 7 months, in which the chronic myelomonocytic leukemia (CMMoL) was 7 months, and 4 months for each of the refractory anemia with excess of blasts (RAEB) or the refractory anemia with excess of blasts in transformation (RAEB-T), however, the median survival had not been reached at 27 months for refractory anemia (RA) and at 33 months for refractory anemia with ring sideroblasts (RARS). Low-does arabinosyl cytosine (Ara-C) was administered in 9 patients with RAEB and RAEB-T, but no survival benefit was noted. Infection, especially pneumonia, was the most common cause of death. In 61 febrile episodes with clinically suspected sepsis, 10 (17%) were documented to associate with bacteremia. Twelve patients (7 RAEB, 4 RAEB-T, and 1 CMMoL) evolved to acute myelogenous leukemia (AML), the median interval from diagnosis to evolution was 4.8 months. This series indicates that only two groups of FAB subtypes could be clearly separated in terms of morphological findings and clinical outcome; RA and RARS constitute a good prognostic group, whereas RAEB, CMMoL, and RAEB-T constitute a poor prognostic group.
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PMID:Primary myelodysplastic syndrome: an analysis of 56 patients. 146 34

We conducted a phase II evaluation of edatrexate in 17 previously untreated patients with advanced adenocarcinoma of the pancreas; 14 patients had at least one month of therapy. The initial dose was 80 mg/m2iv. Treatment was administered weekly for 5 weeks, then every other week. Toxicity was generally mild. The median WBC nadir was 5.4 (range 0.6-7.4) x 10(3)/microliters, and the median platelet nadir was 164.0 (range 62.0-341.0) x 10(3)/microliters. One patient died with sepsis and gastrointestinal bleeding associated with pancytopenia. Five patients had a mild rash. Nausea occurred in 6 patients, including 3 who had vomiting. In addition, 11 patients complained of vague malaise which seemed to begin within 24-48 hours after administration of edatrexate, and lasted for 2 to 3 days, resolving within 6 days of drug administration. Median survival was 85 days. Although 5 patients had stable disease, including one with relief of pain, no major responses were seen, excluding, with 95% confidence, a response rate in excess of 20%.
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PMID:Phase II trial of edatrexate in patients with advanced pancreatic adenocarcinoma. 148 6

We studied 12 children who presented with a recently recognized syndrome. The salient features of this new syndrome were recurrent fever; hepatosplenomegaly; pancytopenia; blond, golden to silvery gray hair; hypopigmented skin, progressive white matter demyelination; and early death. Seven patients died, four with severe central nervous system (CNS) involvement, and three with bone marrow failure and sepsis. Cutaneous anergy to recall antigens was present in all patients. Other immunological abnormalities were poor antibody responses; deficient T-cell responses to phytohemagglutinin (PHA), concanavalin A (Con-A), and allogeneic lymphocytes; hyperresponsiveness of B lymphocytes to pokeweed mitogen; and variable phagocytic defects. Histopathologic examination of the hair and skin biopsies showed characteristic distribution of melanin with melanocytes present in normal numbers but with fewer short dendritic processes. Langerhans' cells were present in normal numbers in some patients and sparse in others. This syndrome seems to cluster into two tribes from two different geographical areas in the Arabian Peninsula. In the eight families studied, 12 other siblings and close relatives were found to be affected. The mode of inheritance in this syndrome is that of an autosomal recessive pattern. We propose the term "PAID syndrome" to identify patients with the above features.
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PMID:Partial albinism, immunodeficiency, and progressive white matter disease: a new primary immunodeficiency. 149 Jun 25

The case is a 45-year-old female who underwent right total nephroureterectomy and partial cystectomy for renal pelvic cancer. During the operation, she received blood transfusion. On the 10th postoperative day, she developed high fever and skin rush on the face, which were followed by liver dysfunction and pancytopenia. On the 18th post-operative day, she died of sepsis. Autopsy revealed hypoplasia and aplasia of the bone marrow and severe atrophy of the systemic lymph nodes and spleen. The characteristic clinical course and autopsy findings of this case closely resembled graft-versus-host disease which is observed after bone marrow transplantation or blood transfusion given to patients with severe immunodeficiency. It is therefore strongly suggested that postoperative erythroderma of this case was induced by graft-versus-host reaction due to blood transfusion given during the operation.
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PMID:[An autopsy case of postoperative erythroderma after nephroureterectomy possibly induced by graft-versus-host reaction following blood transfusion]. 153 97

We report a case of fatal transfusion-associated graft versus host disease (GVHD) that developed in a patient with laryngeal cancer. After a 39.6Gy irradiation dose, total laryngectomy with right radical neck dissection was performed. The postoperative course was uneventful. However, seven days after blood transfusion, high fever (38.5 degrees C) suddenly appeared. On the ninth day, watery diarrhea and facial erythema were observed. On the 12th day, liver disturbance and pancytopenia developed. The patient died on the 16th day because of overwhelming sepsis. Transfusion-associated GVHD has a mortality rate of more than 90%. Therefore, the most important procedure for preventing GVHD is the use of irradiated blood products. Furthermore unnecessary blood transfusion should be avoided.
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PMID:[A case of transfusion-associated GVHD after total laryngectomy]. 163 93


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