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)

A 88-year-old man was admitted because of the left chest pain due to herpes zoster for 1 week. Blood analyses and immunoelectrophoresis revealed anemia, severe neutropenia, rouleaux formation and IgM, lambda-type monoclonal gammopathy. The HE staining and peroxidase-anti-peroxidase staining of biopsy specimens of the cervical lymph node swelling appeared from the fifth hospital day, revealed an increase in atypical lymphocytes bearing IgM, lambda-type immunoglobulin. Then a diagnosis of primary macroglobulinemia was made. Although the patient's clinical findings transiently improved after chemotherapy with prednisolone and vindesine, he died of a septic shock which appeared after klebsiella pneumonia and sepsis. We reported an unusual case of primary macroglobulinemia with severe neutropenia, leading to a rapid development of septic shock after the chemotherapy.
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PMID:[Primary macroglobulinemia with severe neutropenia, leading to a rapid development of septic shock]. 249 64

Scleromyxedema is a rare fibromucinous connective tissue disorder characterized by papular skin lesions associated with sclerosis and a serum monoclonal gammopathy. Little is known about either the natural history or the systemic manifestations of this disease. We reviewed the medical records of 19 patients with biopsy-proven scleromyxedema seen from 1950 to 1985 for evidence of systemic disease. There were 10 males and 9 females with a median age at diagnosis of 53 years. Monoclonal gammopathy was present in 13 patients. Eight patients complained of dysphagia; 3 had proximal esophageal dysfunction and 1 had total esophageal aperistalsis on barium swallow. Proximal muscle weakness was noted in 5, with an inflammatory myopathy in 3. Six patients complained of dyspnea on exertion. Of these, 5 had reduced diffusing capacity, 3 had reduced volumes, and 2 developed cor pulmonale. Pathologic changes characteristic of "scleroderma kidney" were demonstrated in 1 patient at postmortem. One patient had Raynaud's phenomenon and 2 had arthralgias/arthritis with noninflammatory synovial fluids. Although 8 of 12 patients treated with melphalan noted regression of their skin changes, no consistent improvement in the extracutaneous manifestations was demonstrated. Furthermore, 2 patients died of sepsis related to melphalan-induced myelosuppression, and 4 developed hematological malignancies following melphalan therapy. In conclusion, systemic manifestations in scleromyxedema are more prevalent than previously recognized, and can resemble those of scleroderma. Significant toxicity occurred with the use of alkylating agents in these patients, with treatment-related complications developing in 45% of patients treated with melphalan. The lack of definitive data regarding the natural history of this disease complicates the question of optimal therapy, but the use of alkylating agents should be reserved for those patients with severe debilitating skin disease.
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PMID:Scleromyxedema: a scleroderma-like disorder with systemic manifestations. 333 81

We reported a 74-year-old woman with plasmacytoid lymphocytic lymphoma who initially presented with exertional dyspnea, conscious disturbance, ascites, and lytic skull lesions. Protein electrophoresis and immunoelectrophoresis showed monoclonal IgG-lambda gammopathy with IgG level of 13300 mg/dl and marked suppression of the residual normal immunoglobulins. Abdominal computed tomography (CT) revealed a pattern mimicked cancerous peritonitis. She responded to plasmapheresis with much clinical improvement of the hyperviscosity syndrome but died of H. influenza sepsis 3 weeks after diagnosis. The unusual and interesting features of this case included: (1) IgG hyperviscosity syndrome and diffuse peritoneal involvement as the initial manifestations in plasmacytoid lymphocytic lymphoma, and (2) presence of lytic bone lesions in conjunction with high level of M--component and marked suppression of normal residual immunoglobulins in a patient with lymphoma rather than multiple myeloma.
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PMID:Plasmacytoid lymphocytic lymphoma presenting with IgG hyperviscosity syndrome and peritoneal lymphomatosis. 764 Nov 9

A 78-year-old man was diagnosed as leukocytosis in February 1994. Physical examination revealed marked hepatosplenomegaly. A peripheral blood examination disclosed 95,090/microliter leukocytes without hiatus leukemicus, 6.5 g/dl Hb, and 15.0 x 10(4)/microliter platelets. The neutrophil alkaline phosphatase score was 27, and serum VB12 was above 1,600pg/ml. IgG was identified as monoclonal immunoglobulin of type lambda. Bone marrow specimens demonstrated marked granulocytic hyperplasia. Neither the Philadelphia chromosome (Ph1) nor BCR gene rearrangement was detected; hence, the diagnosis of Ph1 (-) chronic myeloid leukemia (CML) was made. The patient was treated with hydroxyurea and low-dose VP-16 with no improvement, and died of pneumonia and sepsis in June 1995. This case was considered to be consistent with atypical CML (aCML) according to the FAB classification because monocytosis was not observed. It seems likely and interesting that the coexistent monoclonal gammopathy and aCML might have arisen from common abnormal hematopoietic stem cells.
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PMID:[Atypical chronic myeloid leukemia presenting with trilineage dysplasia and IgG (lambda) type monoclonal gammopathy]. 1019 7

Splenic rupture (SR) is a rare adverse event observed in patients treated with G-CSF as a peripheral hematopoietic stem cell (PHSC) mobilizing agent, mostly in myeloma multiple and amiloidosis; to date, to our knowledge, it has not been previously described in plasma-cell leukemia (PCL). We report a case of a woman with PCL, who presented a SR after PHSC mobilization with Cyclophosphamide+G-CSF. The spleen removed showed hematopoietic foci and amiloid material. In the course of a second mobilization, 2 months after, the patient died from sepsis. We considered it important to report this case, in order to keep in mind the possibility of SR in patients with malignant gammopathy.
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PMID:Splenic rupture in a plasma cell leukemia, mobilized with G-CSF for autologous stem cell transplant. 2081 17

The features of multiple myeloma are due to plasma cell proliferation and the production of a monoclonal antibody. Skeletal disease with hypercalcemia, bone marrow dysfunction, sepsis, and renal failure are the commonest problems noted. The diagnosis is suggested by the demonstration of monoclonal gammopathy in the serum or the presence of free immunoglobulin light chains in the urine. Other diagnostic features are bone marrow plasmacytosis, hypogammaglobulinemia and lytic bone lesions. Beneficial responses are seen with supportive care of complications, radiotherapy for severe bone disease and chemotherapy to decrease the tumor burden.
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PMID:Multiple myeloma. 2127 78

Serum protein electrophoresis is commonly used in case of acute or chronic renal failure. It can lead to the etiologic diagnosis by detecting monoclonal gammopathies which are frequently complicated by renal failure, such as cast nephropathy, Randall's disease or amyloidosis, or to explore an associated inflammatory syndrome. We report the occurrence of two monoclonal components in a patient without any monoclonal component 10 days earlier. The sudden appearance of these two monoclonal components associated to the context of sepsis of urinary origin suggested the diagnosis of transient monoclonal gammopathy. This hypothesis was confirmed by monitoring serum protein electrophoresis that showed a gradual decrease of these two monoclonal components few weeks after the resolution of the infectious disease. The main etiological factors of transient monoclonal gammopathies are infectious or autoimmune diseases. In this context, it is important to delay the achievement of serum protein electrophoresis after the acute episode, in order to avoid to falsely conclude to hematologic malignancy diagnosis. This can prevent costly biological examinations of these transient monoclonal gammopathies and invasive procedures like bone marrow examination.
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PMID:[An acute monoclonal gammopathy?]. 2584 41

Clarkson's syndrome, also known as idiopathic systemic capillary leak syndrome, is characterised by vascular hyperpermeability resulting in intravascular hypovolaemia and shock. A clinician should consider the diagnosis if other causes of shock, for example, sepsis and anaphylaxis, are ruled out and concomitant hyperviscosity is not caused by a myeloproliferative disease. Here, we describe a patient presenting with severe plasma leakage and assumable blood hyperviscosity leading to splenic infarction, gastrointestinal ischaemia-reperfusion syndrome and transient dysarthria. Our patient was first suspected of polycythaemia vera and phlebotomies were performed. Awareness of this syndrome and subsequent correct treatment is essential to prevent complications and to reduce mortality. As in our patient, most patients with Clarkson's syndrome have a monoclonal gammopathy, light-chain-type kappa. Prophylactic treatment with intravenous immunoglobulin (IVIg) is advised to prevent recurrence of capillary leak. Our patient did not suffer from another symptomatic episode after starting IVIg.
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PMID:Hyperviscosity-related splenic infarction, gastrointestinal ischaemia-reperfusion injury and transient dysarthria in a patient with distributive shock due to idiopathic systemic capillary leak syndrome (Clarkson's syndrome). 3198 Apr 75