Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty patients with advanced acute leukemia and lymphoma were treated with the sequential combination of high dose ARA-C (HiDAC 3 gm/m2 infused i.v. over 3 h at 0, 12, 24, 36 h) and asparaginase (ASP 6.000 IU/m2 i.m. at hour 42). The sequence was given on day 1 and 8 irrespective of the degree of myelosuppression. Of 22 patients with leukemia there was only one who was absolutely refractory to therapy. Complete remission was induced in 3 patients with ANLL (30%) and in 3 with ALL (30%). Three patients became hypoplastic but recovered with blasts and 12 died from infection, complicated by intracranial hemorrhage in 3, during hypoplasia. Of 8 patients with lymphoma, 2 were clearly refractory to therapy, one died from sepsis and the remaining 5 all entered remission (2 CR + 3 PR, 62%). Activity of HiDAC/ASP against CNS disease is suggested by the clinical response seen in patients with overt meningeal or intracerebral involvement. Toxicity associated with HiDAC/ASP was mainly hematologic. All but one patient experienced hypoplasia and severe pancytopenia; documented infections and major hemorrhages occurred in 80 and 20% of patients respectively. We conclude that HiDAC/ASP is a regimen with definite activity against acute leukemia and lymphoma including CNS disease. Alternate treatment schedules should be explored in order to reduce marrow toxicity.
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PMID:Sequential combination of high dose ARA-C (HiDAC) and asparaginase (ASP) for the treatment of advanced acute leukemia and lymphoma. 647 2

Eleven patients with familial hemophagocytic lymphohistiocytosis (FHLH) are described. They all belonged to four Jewish families of Iranian and Iraqi origin. Parental consanguinity was found in three families. The age of onset of disease ranged from 6 weeks to 36 months. All patients had fever, wasting, and enlargement of the liver and spleen. In addition, lymph-node enlargement and neurologic complications were common. The most consistent laboratory findings were pancytopenia, atypical lymphomonocytoid cells in the peripheral blood, abnormal liver function test results, and increased cerebrospinal fluid protein. The course was fatal in all patients. Nine of the 11 patients died within 2 weeks to 3 months of presentation, and 2 patients achieved temporary remissions but died of disease within 8 and 24 months, respectively. Response to antibiotic therapy or to the administration of corticosteroids and cytotoxic drugs was unimpressive. Pancytopenia complicated by sepsis or bleeding, hepatic failure, or encephalopathy were the terminal events. This report draws attention to the existence of FHLH in Jews of Iranian-Iraqi origin in whom parental consanguinity is very common.
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PMID:Familial hemophagocytic lymphohistiocytosis (FHLH) in Israel. I. Description of 11 patients of Iranian-Iraqi origin and review of the literature. 648 37

Histiocytic medullary reticulosis (HMR) was originally defined as a neoplastic disorder. Some cases reported as HMR have been characterized by a systemic proliferation of mature histiocytes showing hemophagocytosis, bone marrow necrosis, pancytopenia, hepatitis, and coagulopathy. Clinically, these patients have fever and constitutional symptoms and often have hepatosplenomegaly and lymphadenopathy. Although there is a high mortality rate, this process appears to be reactive and has been associated with active viral infection. Similar cases have been briefly described that were associated with other agents or disease processes, but concomitant viral infections were not excluded. Three characteristic examples of this hemophagocytic syndrome that were associated with bacterial sepsis are described. Active infection by those viruses that have previously been associated with the syndrome was excluded. It appears that the hemophagocytic syndrome may be associated with various types of active disseminated infections.
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PMID:Bacteria-associated hemophagocytic syndrome. 649 70

The morphologic and clinical features of four patients who developed significant bone marrow and blood dyspoiesis after successful chemotherapy for acute nonlymphocytic leukemia (ANLL) are described. This postleukemic dyspoiesis developed 1-6 months after leukemia induction therapy and persisted for 5-20 months in a relatively stable state. This period of prolonged dyspoiesis was not associated with rising myeloblast counts or clinical evidence of relapse. Dyspoietic abnormalities developed while two patients were receiving maintenance chemotherapy; the other two patients received no maintenance therapy. The dyspoietic changes in these four patients greatly exceeded those noted in a control group of ANLL patients on maintenance chemotherapy. The morphologic features of postleukemic dysmyelopoiesis were similar to those described in preleukemic dysmyelopoietic disorders. Erythroid abnormalities included hyperplasia with ring sideroblasts, megaloblastic changes, and cytoplasmic PAS reactivity. Myeloid abnormalities consisted of left-shifted granulopoiesis with hyper- and hyposegmentation; megakaryocytic abnormalities included hyperplasia with a predominance of hypolobulated forms. Three of the four patients eventually suffered relapse and have died. The fourth patient died of sepsis after 20 months of pancytopenia and dysmyelopoiesis. Theories to explain the development of postleukemic dysmyelopoiesis are presented which emphasize the possibility of drug-induced leukemia cell differentiation. Cytogenetic studies will be necessary to establish any relationship between ANLL and the subsequent postleukemic dysmyelopoiesis.
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PMID:Postleukemic dysmyelopoiesis. 665 Apr 93

The patient was a 61-year-old man who initially complained of skin pigmentation. He was diagnosed as malignant acanthosis nigricans associated with gastric cancer (IIc advanced) in March 1981. Surgical excision of the stomach was performed in May 1981. In July 1981 he experienced headache, vomiting and muscle weakness. A diagnosis of meningeal carcinomatosis was made based on his clinical symptoms, spinal fluid examination and a computerized tomography scan. The intraventricular administration of cytosine arabinoside, methotrexate and hydrocortisone via the Ommaya reservoir resulted in complete remission. In November 1981 the patient died of bronchopneumonia and sepsis due to pancytopenia.
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PMID:[Case of gastric cancer associated with acanthosis nigricans and meningeal carcinomatosis]. 688 26

A teenage white male was recently evaluated for a rapidly enlarging mass in the right groin. Biopsy of the mass disclosed Burkitt's lymphoma. Aggressive therapy was instituted, including extirpative surgery, followed by chemotherapy and radiation therapy. Using these combined modalities, it was possible to eliminate the lymphoma. Unfortunately, severe pancytopenia and immunosuppression developed and the patient died of gram-negative sepsis. This disease should be considered in the differential diagnosis of any rapidly enlarging solid tumor in a young patient.
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PMID:Burkitt's lymphoma in the femoral triangle of a white american male. 707 28

After perioperative adjuvant chemotherapy of a sigma-adenocarcinoma with 400 mg peptichemio and 500 mg 5-fluorouracil a 61-year-old woman developed a severe intoxication: myelosuppression with pancytopenia, gastroenteritis and ulcerative proctitis, toxic hepato- and myocardiopathy, impaired renal function and alopecia. As a result of reduced resistance pneumonias, urinary tract infection, sepsis, cytomegaly infection and candidiasis of the oral mucosa occurred. The toxic effects are attributed mainly to the high dose of peptichemio.
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PMID:[Severe intoxication after combined chemotherapy of a sigma-adenocarcinoma with peptichemio and 5-fluorouracil (author's transl)]. 711 29

Identical twin Caucasian boys, age 3 months, were seen with fever of unknown origin, hepatosplenomegaly, and pancytopenia. The diagnosis of familial erythrophagocytic lymphohistiocytosis (FEL) was suspected after examination of Twin A's bone marrow and confirmed by an open liver biopsy of Twin B. Twin A died shortly after diagnosis despite treatment with vincristine and prednisone. At autopsy, the diagnosis was confirmed. Twin B responded initially to a three-week course of weekly vincristine and daily prednisone, but symptoms soon recurred. In an effort to enhance delivery of chemotherapy to the active macrophage target, platelets were loaded with vinblastine and then administered intravenously to th patient every 7-10 days. There was an encouraging response reflected by the disappearance of symptoms and the return of peripheral blood count to the normal range, although increased number of histiocytes was still demonstrable in his bone marrow. After nine weeks, he lapsed completely and became refractory to treatment. He died of pseudomonas sepsis four months after diagnosis. This is the first known attempt to deliver a chemotherapeutic agent directly to the macrophages in treating this disease and represents an interesting concept that merits further exploration.
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PMID:Familial erythrophagocytic lymphohistiocytosis: treatment with vinblastine-loaded platelets. 719 95

A 67-year-old woman was treated with MP-P therapy and combination chemotherapy for multiple myeloma IgG-lambda type. After the therapy for about three years, pancytopenia developed. Bone marrow aspiration study revealed a few of myeloma cell and many atypical cells showing promyelocytic feature. Chromosomal abnormality was 46, X, -X, +8, -13, +mar. CD33 and CD56 were positive, but CD16 and HLA-DR were negative. We diagnosed as multiple myeloma complicated with secondary myeloid/natural killer (NK) cell acute leukemia. After she had been treated with low dose etoposide for leukemia, she obtained complete remission. But since myeloma progressed and the amount of M protein was increased, she was treated with dexamethasone and low dose etoposide, resulting in a decrease in the amount of M protein. After that, because of leukemic cell re-proliferation, she was treated with etoposide. However, she died of sepsis due to severe myelosuppression. This case was interesting one in coexist of multiple myeloma and secondary myeloid/NK cell acute leukemia, and those affecting her clinical course each other.
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PMID:[Secondary myeloid/natural killer cell acute leukemia appeared in multiple myeloma treated with melphalan]. 756 97

Over a follow-up period of ten years, nine of our 100 patients with multiple myeloma (MM), developed myelodysplastic syndrome (MDS, preleukaemia). MDS occurred 19-156 (median 35) months from the diagnosis of MM. Six patients presented with pancytopenia and no patients had active MM at the time of MDS diagnosis. Three patients were defined as having refractory anaemia (RA) and six as refractory anaemia with excess blasts (RAEB) or RAEB in transformation (RAEBT), according to the FAB classification. The clinical course is characterized by increasing red blood cell and platelet transfusion requirements, recurrent infections and bleeding episodes. All patients, except for one, died within 3 to 8 (median 5) months from MDS diagnosis. The causes of death were sepsis or bleeding; three patients underwent leukaemic transformation. Thus, the clinical course of this small group of myeloma patients who developed secondary MDS (sMDS), was similar to other series of patients with sMDS. Serial bone marrow examinations suggest an initial hypercellular phase, followed by a rapidly evolving preterminal hypocellular marrow. In an attempt to detect MM patients at risk of developing sMDS, the epidemiological (including ethnic), clinical and laboratory data of the 9 MDS patients at the time of the MM presentation were reviewed and compared to the other MM patients. No significant differences were observed between the two groups in most parameters, except for two. All MDS patients were Ashkenazi Jews and no patients of Sepharadic origin developed MDS. Also, no IgA-myeloma patient developed MDS. If these findings are confirmed in a larger series, it may point to subgroups at risk which may require a different approach.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Secondary myelodysplastic syndrome in multiple myeloma--a study of nine patients with an attempt to detect myeloma patients at risk. 789 Feb 64


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