Cases reported "Hemorrhage"

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1/23. Importance of basophilia in haematopoietic disorders.

    To the significance of basophilia in haematopoietic disorders, six draw attention to cases have been analyzed. Associated diseases included acute myelogenous leukaemia (AML-M2, M3, M4, and M6), refractory anaemia with excess of blasts (RAEB) and RAEB in transformation (RAEB-T). Two AML cases (M2, M6) were preceeded by myelodysplastic syndromes (MDS). All patients showed greater than 3% basophilia in peripheral blood and bone marrow. basophils were identified successfully by metachromatic staining with toluidine blue in all cases. Three patients (M3, M4, RAEB) presented with lymphadenopathy, suggesting an association with extramedullary involvement. Neutrophil alkaline phosphatase (NAP) activity was significantly reduced in four patients with AML (M2, M3, M4) and RAEB-T. The clinical course was generally unfavourable characterized by short remission duration or disease progression except for the patient with RAEB. Haemorrhage was the main cause of death rather than infection. cytogenetic analysis revealed unique abnormalities involving chromosomes 3q21, 5q31, and 17q11 where the genes for some haematopoietic growth factors or their receptors are located, in addition to t(6;9) and t(15;17).
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2/23. Posthaemorrhagic iron deficiency. Clinical course, 59Fe whole-body iron losses, and oral iron supplementation.

    Clinical and laboratory data characterizing post-haemorrhagic anaemia with still normal iron stores and posthaemorrhagic iron deficiency in the manifest, latent or prelatent stage are presented. Initially, increased 59Fe whole-body iron losses (greater than 0.1-3.6%/day) returned to normal range (less than 0.1%/day) after haemostasis. Subsequently, slow increase of haemoglobin and repletion of iron stores occurred under normal diets. Manifest, latent, and prelatent iron deficiencies were corrected much more rapidly by total doses of 12.0, 10.5 and 8.0 g iron (Fe2 sulfate), respectively, when 2 X 50 mg/day were given in quick-release capsules apart from meals.
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3/23. A tale of two syndromes: ovarian hyperstimulation and abdominal compartment.

    Abdominal compartment syndrome complicated severe ovarian hyperstimulation in a 35 year old woman with multiple bowel resections due to Crohn's disease. pain from ovarian enlargement necessitated hospital admission. Despite intravenous fluid administration and heparin prophylaxis, ilio-femoral deep vein thrombosis developed. Treatment by intravenous heparin was complicated by repeated intra-ovarian bleeding, anaemia and acute renal failure requiring haemodialysis. Intra-abdominal pressures were elevated. After placement of an inferior vena caval filter and discontinuation of heparin, there was slow spontaneous recovery without surgery.
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4/23. Disseminated fungal infection complicated with pulmonary haemorrhage in a case of acute myeloid leukaemia.

    Pulmonary haemorrhage is a common necropsy finding in acute leukaemia, however, it is rarely diagnosed during life. A man with acute myeloid leukaemia is reported who presented with disseminated fungal infection, anaemia, thrombocytopenia, and subconjuctival and petechial haemorrhages. During the course of the patient's illness, the chest infection was complicated with bilateral pulmonary haemorrhage. The diagnosis of pulmonary haemorrhage was based on characteristic clinical and radiological findings. The patient improved on treatment.
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5/23. Unexpected birth trauma with near fatal consequences.

    Rupturing of the liver due to delivery is an uncommon but severe birth trauma. Although described in the preterm neonate with very low birthweight or after complicated delivery, we present a case of capsular liver haemorrhage in a term newborn with normal birthweight after a seemingly uncomplicated delivery. The infant presented with severe shock and petechiae as first symptoms and initial therapy was based on the hypothesis of sepsis. Clinical suspicion of liver haemorrhage a few hours later was confirmed with abdominal ultrasound. Since shock was not amenable to fluid replacement therapy, the haemorrhage had to be managed surgically. Even without evidence of birth trauma, intra-abdominal bleeding must always be suspected in a newborn with suddenly prevailing shock and unexpected anaemia.
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6/23. gastric antral vascular ectasia--a cause of refractory anaemia in systemic sclerosis.

    Recurrent gastrointestinal haemorrhage is an uncommon manifestation of systemic sclerosis. We report a case of gastrointestinal bleeding due to gastric antral vascular ectasia (GAVE) in a patient with systemic sclerosis. Failure to recognise the condition as a cause of gastrointestinal bleeding may delay the instigation of appropriate treatment. GAVE should be considered in the differential diagnosis of anaemia in patients with autoimmune conditions such as systemic sclerosis and primary biliary cirrhosis.
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7/23. Treatment of life-threatening post-haemorrhagic anaemia with cell-free haemoglobin solution in an adolescent Jehovah's Witness.

    Severe blood loss carries an increased risk of a fatal outcome in patients who do not consent to the use of blood products. For patients who base this decision on a religious belief system, such as the jehovah's witnesses, important distinctions may exist between the appropriateness of various blood products. We describe the first successful use of purified human cell-free haemoglobin solution in a paediatric patient, in this case in the management of life-threatening anaemia in a 14-year-old female Jehovah's Witness with immune thrombocytopenia.
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8/23. Acquired haemophilia in hiv negative, HHV-8 positive multicentric Castleman's disease: a case report.

    Multicentric Castleman's Disease (MCD) is an atypical lymphoproliferative disorder, related to human herpesvirus 8 (HHV-8) infection and often associated with autoimmune diseases such as haemolytic anaemia and thrombocytopenia. Acquired haemophilia (AH) is a rare, life-threatening disease, which can occur in association with lymphoproliferative disorders, although only one case of AH in MCD has been described so far. We report the case of a human immuno deficiency virus negative 71-yr-old woman referred to our hospital for prolonged bleeding on surgical site following a lymph node biopsy. Lymph node histology revealed MCD, while the screening for the bleeding disorder showed prolonged activated partial thromboplastin time (APTT) (ratio: 1.89, normal value <1.24), low factor viii (FVIII:C) levels (6%) with anti-factor viii antibodies (2.3 Bethesda units), leading to a diagnosis of AH. Virological studies on plasma, lymphocyte and bronchoalveolar wash showed positivity for HHV-8 infection. Treatment with steroids (metilprednisolone 1-1.5 mg/kg/d) and cyclophosphamide (100 mg/d orally) was unsuccessful, and then antiviral therapy with cidofovir (5 mg/kg/wk) was started. A transient normalisation of APTT was seen after two administrations of cidofovir, but then coagulation parameters worsened and a large haematoma of the arm appeared. Bleeding was successfully stopped with two boluses of recombinant activated factor VII (Novoseven 90 microg/kg). Therapy with anti-CD 20 monoclonal antibody rituximab (Mabthera 375 mg/m2 once a week for 4 wk) was started, and following two administrations APTT normalised once again. Cardiological and neurological complications arose before the third dose of rituximab and the patient died shortly afterwards.
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keywords = anaemia
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9/23. Defibrination and systemic bleeding caused by an imported African snakebite.

    A 55-year-old man was referred from Burkina Fasso for coagulation disorders with severe spontaneous systemic bleeding. He had been bitten six days before by a snake that could not be definitely identified. No specific treatment had been started in africa. The patient was admitted with severe anaemia, incoagulable blood with undetectable fibrinogen. Multiple haematomas in the chest and abdomen were found at computed tomography. blood transfusions and the administration of fresh frozen plasma and fibrinogen did not result in any clinical or biological improvement. The clinical course was dramatically reversed after the infusion of two vials of Pasteur polyspecific antivenom (Echis-Bitis-Naja). According to the geographical distribution of this snake species, it seems very likely that the snakebite was caused by Echis ocellatus. Even given with delay, the antivenom was effective and well tolerated.
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keywords = anaemia
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10/23. Tailored therapy of adult acute leukaemia in jehovah's witnesses: unjustified reluctance to treat.

    Treatment of acute leukaemia in adult jehovah's witnesses (JW) is challenging because of 'a priori' refusal of most physicians to apply diagnostic and therapeutic procedures to haematological abnormalities resembling acute leukaemia. Rejection of blood transfusions by individuals of this faith is usually blamed to justify this attitude, thus leading to severe personal, medical and psychological distress related to the lack of care. We therefore intended to verify whether a standard (tailored) chemotherapy, without the use of prophylactic blood product transfusions, could be applied during treatment of acute leukaemia under such circumstances. Eleven consecutive JW adult patients with acute leukaemia, all of whom had been denied care in other institutions, were treated at the European Institute of Oncology (EIO) in Milan, italy. Five had acute lymphoblastic leukaemia (ALL) (one bcr/abl positive), six had acute myeloid leukaemia (AML) with immunophenotype and/or cytogenetic intermediate-high risk features, except one patient with acute promyelocytic leukaemia (APML). Standard induction chemotherapy [cytosine arabinoside (ARA-C) and daunorubicin (DNR) for AML, vincristine (VCR), DNR and prednisone (PDN) for ALL, all-trans retinoic acid (ATRA) and DNR for APML] with the antracycline dose of at least 30 mg/sqm were used. All patients experienced severe anaemia after induction chemotherapy despite erythropoietin. Median haemoglobin nadir for patients with ALL and AML was 4.5 g/dL (range 1.3-6.9) and 5.1 g/dL (range 2.6-6.8), respectively. Median platelet nadir counts for all patients was 14.5 x 10(9))/L (range 1-24). One patient died during induction probably due to haemorrhage. Four of five patients with ALL achieved a complete remission (CR) (including the bcr/abl case) while among patients with AML only the one with APML achieved CR. Three patients (APML = 1 and ALL = 2) are still alive and disease-free. This small series of adult patients with leukaemia illustrates difficulties in treating patients who are practising JW, yet nevertheless provides a significant argument against the prejudicial decision leading to evasion of treatment in these patients.
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