Cases reported "Hemolysis"

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1/12. Hereditary spherocytosis with hemolytic crisis during pregnancy. Treatment by splenectomy.

    Hemolytic anemia was detected in 2 young women in the second trimester of pregnancy. The diagnosis of hereditary spherocytosis was made, and the patients underwent uneventful splenectomy. Five similar cases from the literature are reviewed.
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2/12. Interaction of sickle cell trait with hereditary spherocytosis: splenic infarcts and sequestration.

    The association of sickle cell trait (SCT) and hereditary spherocytosis (HS) has been reported in only 18 patients. Three of these 18 patients experienced splenic infarct or acute splenic sequestration. We report here a 46-year-old African-American male, the oldest reported case to date, who experienced episodes of hemolysis and severe left upper quadrant pain for the past 26 years. The patient had compensated hemolysis with splenomegaly. A CT scan of the abdomen revealed a large infarct in the spleen. The diagnosis of SCT was confirmed with isoelectric focusing, cation exchange and reverse-phase HPLC. The presence of a silent, interacting globin variant as the cause of hemolysis and sickling in the spleen was ruled out by sequencing of the alpha1-, alpha2- and beta-globin genes. The diagnosis of HS was established by an osmotic fragility test. The interaction of HS and SCT leads to RBC dehydration with increased MCHC and intracellular Hb S concentration presumably favoring intrasplenic sickling and resultant splenic infarcts and sequestration as seen in this case.
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3/12. Dominant beta-thalassemia with hemoglobin Hradec Kralove: enhanced hemolysis in the spleen.

    We describe a 6-year-old girl and her mother with dominant beta-thalassemia due to hemoglobin Hradec Kralove (Hb HK). Both patients presented microcytic anemia, jaundice, splenomegaly, cholelithiasis, and recurrent hemolytic bouts. Osmotic resistance tests using saline and coiled planet centrifugation revealed the increased fragility of the red cell membrane. On the other hand, the glycerol lysing time was prolonged, and results of the isopropanol test were weakly positive. Despite mimicking the features of hereditary spherocytosis, the results of the genetic analyses verified the second reported family with Hb HK (codon 115, GCC [Ala] --> GAC [Asp]). splenectomy was effective for the amelioration of hemolysis. Of 7 reported patients with Hb variants at beta-globin codon 115 (Hb Madrid and Hb HK), 5 underwent splenectomy. Because of the variable augmentation of extramedullary hemolysis in dominant beta-thalassemias, genotyping is necessary for determining the clinical indication of splenectomy.
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4/12. Recurrent hemolysis-associated pseudoerysipelas of the lower legs in a patient with congenital spherocytosis.

    A 29-year-old patient presented with recurrent erythematous eruptions on both lower legs of 15 years' duration. family history, along with clinical and laboratory examinations, revealed congenital hereditary spherocytosis and excluded other reasons for the erythematous eruptions of the lower legs. During two subsequent episodes, we detected increased hemolysis that disappeared concomittantly on spontanous resolution of the lesions. To our knowledge, this case is the first report showing a recurrent erythematous eruption on the lower legs in a patient with congenital hereditary spherocytosis. These eruptions might be caused by intermittent hemolysis-induced inflammation as a result of the increased osmotic fragility of the erythrocytes and may evolve to chronic leg ulcers later in life.
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5/12. Monoclonal gammopathy in hereditary spherocytosis: a possible pathogenetic relation.

    Two cases of monoclonal gammopathy in patients with hereditary spherocytosis led us to consider the possible pathogenetic relation between these two disorders. Twelve adult patients with hereditary spherocytosis had significant hypergammaglobulinemia in comparison to normal subjects. Retrospective analysis of previous illness in 140 patients with multiple myeloma showed a significant association between IgA myeloma and previous gallbladder disease. We propose that the chronic reticuloendothelial stimulation due to extravascular hemolysis, possibly potentiated by the inflammation associated with cholelithiasis and cholecystitis, may foster neoplastic transformation of immunocytes in patients with hereditary spherocytosis, ultimately leading to the development of monoclonal gammopathy.
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ranking = 1.4
keywords = spherocytosis
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6/12. Haemolysis in athletes due to hereditary spherocytosis.

    Three athletes, one cross-country skier and two middle distance runners, experiencing poor response to intensified training and decline in performance during prolonged periods of frequent competitions, associated with a tendency to develop anaemia, were found to have spherocytosis, and increased osmotic fragility of the red blood cells. All had family members with the same blood abnormality, but without symptoms. The observations suggest that the frequency of hereditary spherocytosis is higher than indicated by overtly affected cases and that mild cases are easily overlooked. Hereditary spherocytosis should be looked for in athletes with a tendency to decline in blood haemoglobin concentration during periods of intensive training and competition.
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7/12. Haemolytic anaemia of pregnancy.

    Haemolytic anaemia apparently induced by pregnancy is reported in an otherwise healthy woman, with a negative family history. Folate deficiency, haemoglobinopathies, enzymopathies, hereditary spherocytosis, paroxysmal nocturnal haemoglobinuria, interstitial haematomata, hypertensive toxaemia, auto-immunity and infections were excluded. The autohaemolysis test showed increased lysis in the presence of added glucose. This abnormality was corrected by insulin when excess glucose was present, but not by insulin alone. It is suggested that this patient's erythrocytes may have been altered by placental lactogen and/or prolactin in such a way as to make them abnormally dependent on insulin for the uptake of glucose.
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ranking = 0.2
keywords = spherocytosis
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8/12. Hereditary spherocytosis with normal osmotic fragility after incubation. Is the autohemolysis test really obsolete?

    Two patients in two families had hereditary spherocytosis but lacked a population of RBCs with increased osmotic fragility after incubation. The diagnosis in each patient was confirmed by the presence of splenomegaly, spherocytosis, reticulocytosis, and abnormal autohemolysis corrected by glucose. sodium flux studies showed increased sodium permeability of the RBC membrane in one patient and normal permeability in another. Hereditary spherocytosis was also present in three other family members of patient 2. The autohemolysis test is of value in confirming the diagnosis in patients with hereditary spherocytosis and normal incubated osmotic fragility.
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ranking = 1.6
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9/12. glucose-induced hemolysis of spheric red blood cells in hereditary spherocytosis: new aspects of the autohemolysis test.

    Increased autohemolysis with a partial protecting effect of added glucose is a common finding in hereditary spherocytosis (HS). For unknown reasons, in some rare cases, glucose fails to prevent the increased autohemolysis (Type II autohemolysis). The authors investigated the autohemolysis of such a patient and found that glucose actually induced the hemolysis prior to energy depletion. Old erythrocytes proved to be more fragile in the presence of D-glucose than did young ones. Other D-hexoses reacted similarly to D-glucose, while L-glucose did not. After being splenectomized, the patient's red blood cells' specific sensitivity to D-hexoses disappeared unless a 24-hour preincubation of his whole blood was performed. Other HS blood samples also became fragile in the presence of D-glucose if a 24-hour preincubation at 37 degrees C was made. Normal blood samples preheated to 50 degrees C or normal washed red blood cells in hypotonic conditions also showed a glucose-induced hemolysis. The authors assume that during the routine autohemolysis test, the accumulation of the hexose is followed by volume expansion and results in hemolysis of those cells with extremely low surface area-to-volume ratio.
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10/12. Interaction of hereditary spherocytosis and alpha thalassaemia: a family study.

    We report here a family of patients with coexistent hereditary spherocytosis and alpha-thalassaemia. The different clinical presentations of the family members were affected by the severity of the alpha-thalassaemia. The haemolytic effect of hereditary spherocytosis was modulated by the increased osmotic resistance of thalassaemia in the patients with both disorders. Coexistence of haemoglobin H disease and hereditary spherocytosis resulted in an asymptomatic state. In contrast, coexistence of alpha-thalassaemia trait and hereditary spherocytosis was still haemolytic. The different shapes of the osmotic curves in the family members correlated with the severity of their symptoms.
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ranking = 1.6
keywords = spherocytosis
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