1/78. Elevated reticulocyte count--a clue to the diagnosis of haemolytic-uraemic syndrome (HUS) associated with gemcitabine therapy for metastatic duodenal papillary carcinoma: a case report.In adults, the haemolytic-uraemic syndrome (HUS) is associated with probable causative factors in the minority of all cases. Cytotoxic drugs are one of these potential causative agents. Although metastatic cancer by itself is a recognized risk-factor for the development of HUS, therapy with mitomycin-C, with cis-platinum, and with bleomycin carries a significant, albeit extremely small, risk for the development of HUS, compared with all other cytotoxic drugs. Gemcitabine is a novel cytotoxic drug with promising activity against pancreatic adenocarcinoma. We are reporting on one patient with metastatic duodenal papillary carcinoma developing HUS while on weekly gemcitabine therapy. The presenting features in this patient were non-cardiac pulmonary oedema, renal failure, thrombocytopenia and haemolytic anaemia. The diagnosis of HUS was made on the day of admission of the patient to this institution. Upon aggressive therapy, including one single haemodialysis and five plasmaphereses, the patient recovered uneventfully, with modestly elevated creatinine-values as a remnant of the acute illness. Re-exposure to gemcitabine 6 months after the episode of HUS instituted for progressive carcinoma, thus far has not caused another episode of HUS.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
2/78. Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantile haemangiomatosis of spleen and pancreas.Diffuse infantile haemangiomatosis of the spleen is a very rare lesion. Large haemangiomas may cause trapping of platelets and coagulation disorders known as Kasabach-Merrit syndrome. We here report the case of an infant with splenic and pancreatic haemangiomatosis presenting with life-threatening thrombocytopenia, anaemia and intravascular coagulation. diagnosis was hampered by reactive erythroblastosis and non-conclusive radiological findings. While treatment with corticosteroids was ineffective, administration of antithrombin iii improved coagulation parameters. After splenectomy the child recovered promptly and has remained free of disease for 3 years to date. CONCLUSION: Occult visceral haemangiomatosis without visible cutaneous haemangiomas should be included in the differential diagnosis of thrombocytopenia, anaemia and consumption coagulopathy. antithrombin iii treatment may be considered to overcome bleeding problems in patients with Kasabach-Merrit syndrome.- - - - - - - - - - ranking = 6keywords = thrombocytopenia (Clic here for more details about this article) |
3/78. Rapid development of severe copper deficiency in a patient with Crohn's disease receiving parenteral nutrition.A 32-year-old man with active Crohn's disease and recurrent small bowel strictures underwent abdominal surgery and was subsequently given total parenteral nutrition (TPN). Severe cholestasis developed and copper was removed from the TPN. Although serum ceruloplasmin levels were within normal limits, 8 weeks after copper removal, he developed pancytopenia. serum copper levels were severely depressed. bone marrow biopsy was consistent with copper deficiency; cytoplasmic vacuolization of both myeloid and erythroid precursors, megaloblastic erthropoiesis, and marked hypocellularity were observed. IV replacement with copper sulfate resulted in improvement in the patient's anemia, neutropenia, and thrombocytopenia, but the patient died suddenly from cardiac tamponade. Postmortem examination revealed fibrinous and hemorrhagic pericarditis. Despite the rare occurrence of overt copper deficiency, this case emphasizes the need to recognize copper deficiency as an important etiology of iron-resistant anemia in patients receiving TPN. Furthermore, the relative rapidity with which our patient developed pancytopenia suggests that, in view of the established recommendation that copper be removed from TPN in cholestatic conditions, serum copper levels must be measured periodically.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
4/78. Massive fetomaternal hemorrhage: how long should children with good evolution be controlled? A case report.We report on a term infant with a severe fetomaternal hemorrhage that caused a serious anemia that was surmounted after several transfusions. After the initial complications, such as persistent pulmonary circulation, severe anemia and thrombocytopenia, the outcome was good. We discuss the importance of a long-term follow-up of affected children, as well as their mothers. No clear parameters for a real prognosis are available. A follow-up is needed in order to detect possible complications in neurological development.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
5/78. Favourable effect of chemotherapy on clinical symptoms and human herpesvirus-8 dna load in a patient with Kaposi's sarcoma presenting with fever and anemia.The case of a patient infected with human immunodeficiency virus type 1 (hiv-1) with Kaposi's sarcoma who presented with fever of unknown origin, severe anemia, thrombocytopenia and hypoalbuminemia but only limited involvement of the skin is presented. Chemotherapy directed at Kaposi's sarcoma resulted in resolution of these clinical signs and symptoms and was associated with a significant reduction in human herpesvirus-8 dna load in serum, despite continued hiv-1 replication. Such a decreasing human herpesvirus-8 load following Kaposi's sarcoma-directed chemotherapy has not been reported previously. These findings suggest that Kaposi's sarcoma was indeed responsible for the clinical syndrome and that this neoplasm is a source of human herpesvirus-8 virus particle production, which can be inhibited by chemotherapy-induced reduction in tumor burden.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
6/78. in vitro inhibition of hematopoiesis in a patient with systemic sclerosis treated with D-penicillamine.A 66-year-old Japanese woman with severe scleroderma developed anemia and thrombocytopenia due to D-penicillamine (D-Pen) treatment, although the leukopenia was not markedly severe. Cessation of D-Pen and the start of corticosteroid therapy were followed by recovery from bicytopenia. We examined the in vitro inhibition of the clonogenic capacity of bone marrow hematopoietic progenitor cells of this patient by D-Pen, and found strong inhibition in burst-forming unit-erythroid and colony-forming unit-megakaryocyte assays, but not in colony-forming unit-granulocyte/macrophage assays. These findings suggest that bicytopenia in this patient was caused by D-Pen and may be due to different sensitivities in the hematopoietic lineage.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
7/78. splenectomy may improve the glomerulopathy of type II mixed cryoglobulinemia.Many patients with type II mixed cryoglobulinemia have been shown to be infected with hapatitis C virus (HCV). Therefore, interferon-alfa has become the first choice of treatment for patients with HCV-associated cryoglobulinemia. However, the disease often relapses after the discontinuation of interferon therapy. The long-term effect of interferon therapy is controversial. Therefore, a more effective therapy needs to be developed. A 62-year-old Japanese woman was admitted to our hospital for the examination of abnormal liver function tests, severe edema, and purpura in her lower extremities. Glomerulopathy secondary to HCV-related cryoglobulinemia was suspected. Her serum creatinine was increased to 2.1 mg/dL. Interferon therapy was considered initially. However, because of pancytopenia caused by liver cirrhosis and splenomegaly, splenectomy was performed in February 1997, before the start of interferon therapy. Renal biopsy specimen taken at the time of the splenectomy showed typical cryoglobulinemic glomerulonephritis. Gradually, after surgery, the patient's thrombocytopenia and anemia improved, her proteinuria and hematuria were decreased, her cryocrit dropped from 15% to 5%, the Ccr increased from 21.1 mL/min to 48.8 mL/min, and the purpura in her lower extremities disappeared. A repeat renal biopsy performed in May 1998 showed marked histological improvement. splenectomy is not widely accepted as a treatment for cryoglobulinemia. Our case suggests the possibility that the monoclonal-IgM component of the type II cryoglobulin may be formed in the spleen. In conclusion, splenectomy may be an effective therapy for cryoglobulinemia in patients with HCV-positive liver cirrhosis and pancytopenia secondary to splenomegaly.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
8/78. neutropenia and anaemia due to carbimazole-dependent antibodies.carbimazole-dependent antibodies to erythrocytes were detected in the sera of three anaemic patients who had been treated with carbimazole for hyperthyroidism. By the use of Rhnull-typed erythrocytes, we could show that some of these were directed against the proteins of the Rh complex. carbimazole-dependent antibodies eluted from erythrocytes showed no binding to other blood cells. One patient also presented with neutropenia and mild thrombocytopenia. Additional carbimazole-dependent antibodies against the neutrophil-specific Fcgamma receptor IIIb (FcgammaRIIIb, CD16b) and the broadly expressed platelet endothelial cell adhesion molecule 1 (PECAM-1; CD31) were detected in this patient's serum. Surprisingly, the PECAM-1-reactive drug-dependent antibodies were also detectable in the sera of the other two patients with normal leucocyte and platelet counts. We assume that carbimazole can induce cell lineage-specific drug-dependent antibodies that cause cytopenia and also drug-dependent antibodies against the broadly expressed PECAM-1 molecule that may cause mild but not severe cytopenia.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
9/78. Intratumoral consumption of indium-111-labeled platelets in a child with splenic hemangioma and thrombocytopenia.The authors report kasabach-merritt syndrome (KMS) in a patient with thrombocytopenia and splenic hemangioma. A 13-month-old boy with a history of anemia, thrombocytopenia, and abdominal mass was admitted to the hospital. The scintigraphic studies showed that a large mass contiguous to the spleen was responsible for the platelet uptake. After partial splenectomy, the platelet count returned to normal. This report of KMS in a child with splenic hemangioma suggests that the scintigraphic studies are mandatory to confirm diagnosis. indium-111-labeled platelets are useful in identifying hemangiomatous sequestration of platelets in patients with thrombocytopenia.- - - - - - - - - - ranking = 7keywords = thrombocytopenia (Clic here for more details about this article) |
10/78. Primary antiphospholipid syndrome presenting with cerebral ischemia, thrombocytopenia, anemia and proteinuria successfully treated with warfarin potassium.A 30-year-old woman with primary antiphospholipid syndrome (PAPS) presented with cerebral ischemia, thrombocytopenia, anemia and proteinuria. Administration of warfarin potassium, without concomitant corticosteroid administration, significantly improved all of these symptoms along with a decrease in the titers of antiCL-beta2-GP-I antibodies and a shortening of prolonged APTT. Therefore, the antiphospholipid antibodies in this patient could have been evoked by vitamin-K-dependent coagulation factors or plasma proteins which are assumed to undergo conformational changes exposing cryptic epitopes. This case report provides clues to the mechanisms underlying the production of antiphospholipid antibodies in patients with PAPS.- - - - - - - - - - ranking = 5keywords = thrombocytopenia (Clic here for more details about this article) |
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