1/26. Littoral cell angioma with severe thrombocytopenia.Littoral cell angioma (LCA) is a recently described splenic vascular tumor. We present a new case in a 62-year-old woman with severe thrombocytopenia and mild bleeding diathesis, but without palpable splenomegaly. Abdominal ultrasound and magnetic resonance showed multiple nodular images, suggesting splenic hemangiomas. A platelet kinetic study revealed a very short platelet survival. As the spleen was the site of platelet destruction, splenectomy was carried out. Histopathological and immunohistochemical data allowed a final diagnosis of LCA. Following splenectomy, the patient showed a transitory normalization of the platelet counts. thrombocytopenia then reappeared but was moderate, without hemorrhagic diathesis. A second platelet kinetic study, performed 16 months post-splenectomy, showed hepatic platelet destruction. However, there were no macroscopic hepatic lesions in a second abdominal magnetic resonance study. This case illustrates the difficulties involved in determining the etiology of many peripheral thrombocytopenias.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
2/26. Acute profound thrombocytopenia without bleeding complications after abciximab administration.Abciximab (c7E3) is the Fab fragment of the chimeric monoclonal antibody directed against glycoprotein IIb/IIIa found on the surface of platelets. It is the first FDA-approved platelet receptor glycoprotein inhibitor. Severe thrombocytopenia (defined as platelet count < 50 x 109/L) on first administration is a rare complication, occurring in only 1.6% of patients in the EPIC (Evaluation of 7E3 for the Prevention of Ischemic Complications) study. Bleeding complications were the rule in these thrombocytopenic patients as reported by EPIC investigators, Kereiakes et al. and Berkowitz et al. Platelet transfusions are required to reverse the bleeding diathesis. Intravenous IgG has not reportedly been helpful in reversing thrombocytopenia, and experience with corticosteroids is not reported in the literature. We report a case of acute profound thrombocytopenia following abciximab administration treated with platelet transfusion augmented by intravenous corticosteroids, in whom there was no bleeding complication.- - - - - - - - - - ranking = 0.5keywords = diathesis (Clic here for more details about this article) |
3/26. A woman with leg cramps and rash.A 68-year-old woman presented with generalized weakness and a rash on her lower extremities. The weakness began one week earlier, shortly after treatment for leg cramps and two days before the rash appeared. She had also had blood-streaked sputum a few days before admission. There was no history of bleeding diathesis, nose bleeding, hematuria, vasculitis, muscle or joint aches, cinchonism, or exposure to tuberculosis. She denied fever, chills, night sweats, leg pain or swelling, chest pain, or abdominal pain.- - - - - - - - - - ranking = 0.5keywords = diathesis (Clic here for more details about this article) |
4/26. Anesthetic implications of the grey platelet syndrome.PURPOSE: To describe the obstetrical anesthetic care provided to two sisters with a rare qualitative platelet disorder, the grey platelet syndrome (GPS). CLINICAL FEATURES: Both patients manifested thrombocytopenia prior to delivery without previous history of a bleeding diathesis or other abnormal laboratory tests of coagulation function. The first required emergency cesarean section due to fetal bradycardia. Due to the thrombocytopenia and the emergency nature of the procedure, general anesthesia was used. During the C-section, 1.5-2 litres of old blood was noted in the abdominal cavity which was attributed to an old splenic capsular tear of unknown etiology. work-up for the thrombocytopenia revealed large platelets on the peripheral smear with abnormal aggregation on platelet function studies. Electron microscopy of the platelets revealed absent alpha granules, diagnostic of GPS. The second patient, the sister of patient #1, presented in a similar fashion. However, at presentation, the platelet count was 112,000 x m(-3) and spinal anesthesia was provided without complication for Cesarean delivery. The same patient presented for a second delivery during which fetal bradycardia necessitated emergency C-section under general anesthesia. Despite administration of six units of platelets, blood loss was 5,200 mL. Her postpartum course was uncomplicated and she and the infant were discharged home on postoperative day #4. CONCLUSION: The primary concerns for the anesthesiologist looking after patients with qualitative platelet defects are related to defective coagulation which influences the need for perioperative replacement of blood products and limits the use of regional anesthesia.- - - - - - - - - - ranking = 0.5keywords = diathesis (Clic here for more details about this article) |
5/26. bernard-soulier syndrome associated with 22q11.2 microdeletion.We describe a Japanese girl with bernard-soulier syndrome and 22q11.2 microdeletion. She had viral infections and recurrent thrombocytopenia and hemorrhagic diathesis after cardiac surgery. As congenital heart defects and abnormal immunity are the most common clinical manifestations associated with 22q11.2 deletion, patients with this association may have a greater risk of developing a severe bleeding disorder.- - - - - - - - - - ranking = 0.5keywords = diathesis (Clic here for more details about this article) |
6/26. Delivery management in a woman with thrombocytopenia of the May-Hegglin anomaly type.thrombocytopenia of the May-Hegglin anomaly type was diagnosed in a woman with no past history of bleeding diathesis, who had been followed during her three pregnancies. No abnormal bleeding occurred although no platelet transfusion was administered during the second and third cesarean sections. Routine platelets transfusion is unnecessary but platelets should be available for use if abnormal bleeding occurs.- - - - - - - - - - ranking = 0.5keywords = diathesis (Clic here for more details about this article) |
7/26. Acute promyelocytic leukemia in childhood. Report of a case with a review of the literature.A rare case of acute promyelcytic leukemia (APL) is reported in a 7-year-old boy. The patient displayed the typical features of APL including impaction of the marrow with promyelocytes, marked elevation of the serum vitamin B12 and transcobalamin I levels and a hemorrhagic diathesis. The bleeding diathesis in the case was due to thrombocytopenia, and there was no evidence for disseminated intravascular coagulation.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
8/26. life-threatening thrombocytopenia associated with acute Epstein-Barr virus infection in an older adult.Acute Epstein-Barr virus (EBV) infection commonly induces hematological abnormalities, most notably atypical lymphocytosis ("infectious mononucleosis"). In addition, mild decreases in platelet counts are commonly encountered in uncomplicated cases; however, severe thrombocytopenia is exceedingly rare. Here, we describe a 58-year-old white man who presented with cervical lymphadenopathy, thrombocytopenia, and a bleeding diathesis with minimal platelet counts of 0.5 x 10(9)/l. The diagnosis of acute EBV was serologically confirmed. Because of the bleeding diathesis and the prior ingestion of aspirin, treatment was started with intravenous methylprednisolone and immunoglobulins. Platelet counts normalized within 7 days, and the patient fully recovered. Although more common in children, adolescents, and young adults, acute EBV infection may also occur in older adults, and this differential diagnosis should be considered in every patient presenting with acute thrombocytopenia. In this report we also briefly summarize the literature on EBV-associated severe thrombocytopenia.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
9/26. Sebastian syndrome: report of the first case in a South American family.The Sebastian syndrome (SS) is a MYH9-related disorders, which are an extremely infrequent group of four autosomal dominant illnesses. SS consist of giant platelets, leukocyte inclusions and thrombocytopenia. To our knowledge, there are no case reports of this syndrome in south america. The propositus was a 35-year-old Argentine woman with a history of purpuric lesions in her lower limbs and thrombocytopenia. Idiopathic thrombocytopenia purpura (ITP) was previously diagnosed, but she did not respond to treatment with steroids. family history failed to provide any evidence of hearing loss, easy bruising, nephritis, renal failure or cataracts. The patient and 11 members of her family were evaluated. The diagnosis of SS was established by demonstrating giant platelets, thrombocytopenia and leukocyte inclusions in peripheral smear in two relatives and by peripheral smear and electronic microscopy in the propositus. MYH9-related disorders should be suspected whenever a patient has a low platelet count or a bleeding diathesis of unknown origin. In these cases, the history, carefully peripheral smear exam, immunocytochemistry and electronic microscopy will be of great help. Differentiation ITP with SS is important to avoid unnecessary diagnostic studies and treatments.- - - - - - - - - - ranking = 0.5keywords = diathesis (Clic here for more details about this article) |
10/26. Fatal thrombocytopenia and thrombocytopathia: an unusual onset of non-small cell lung cancer.BACKGROUND: Although bone marrow is a common site of micrometastases for non-small cell lung cancer (NSCLC), thrombocytopathia and hemorrhagic diathesis are rare causes of death. CASE REPORT: A 57-year-old patient was admitted to the emergency room because of massive nosebleeding and hemoptysis. Routine blood analysis showed thrombocytopenia and prolonged bleeding time; results of functional platelet tests suggested concomitant thrombocytopathia. Routine chest X-ray revealed an 18 mm large spot in the right superior lobe. During the first hour of recovery the patient had another episode of nosebleeding. A bone marrow biopsy showed a wide infiltration with neoplastic cells. histology was compatible with NSCLC. The clinical conditions and hematological parameters progressively deteriorated, and on the third day the patient died because of hypovolemic shock. CONCLUSION: This is a very rare clinical presentation of NSCLC characterized by massive bleeding due to thrombocytopenia and thrombocytopathia secondary to wide bone marrow infiltration.- - - - - - - - - - ranking = 0.5keywords = diathesis (Clic here for more details about this article) |
| | Next -> |