Cases reported "Anemia, Hemolytic"

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1/17. Spherocytosis preceding the development of myelodysplasia.

    We report two patients with prominent spherocytosis in the peripheral blood who later went on to develop myelodysplasia. There was no evidence of a myelodysplastic syndrome at the time of presentation. Morphological abnormalities of the peripheral blood along with possible explanations of spherocytosis are discussed.
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2/17. 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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3/17. Coexistence of hereditary spherocytosis and beta-thalassemia: case report of severe hemolytic anemia in an American black.

    The first reported case of hereditary spherocytosis (HS) and beta-thalassemia in an American black is presented. The diagnosis rested on clinical presentation, family history and specialized laboratory findings. The tendency toward hemolysis of spherocytes and thalassemia red cells may explain the severity of anemia in this patient. The clinical course of these coexistent diseases and the role of splenectomy in relieving the hemolytic component caused by hereditary spherocytosis are described. A detailed review of the literature on HS and beta-thalassemia is also included.
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4/17. The affinity glycated hemoglobin in a family with hereditary spherocytosis and in other non-hemoglobinopathic hemolytic anemias.

    The glycated hemoglobin (GHb) is lowered by hemolytic anemia. The cation-exchange HbA1 has been shown to be lowered by hereditary spherocytosis (HS). The HbA1, however, can be increased by elevations of fetal hemoglobin (HbF). The affinity GHb, a parameter related to, but not identical with, the HbA1, and unaffected by HbF, has been shown to be low in hemoglobinopathies but not, to our knowledge, in HS and other non-hemoglobinopathic hemolytic anemias. Therefore, the affinity GHb and HbF was determined in four members of an HS family and in nine other cases of non-hemoglobinopathic hemolytic anemia, including three autoimmune hemolytic anemias, four red cell fragmentation syndromes (two "Waring blender" syndromes, one thrombotic thrombocytopenic purpura in association with tumor, and one case of disseminated intravascular coagulation), and two red cell membrane defects: paroxysmal nocturnal hemoglobinuria and another case of hereditary spherocytosis. The GHb for these nine cases was 3.6 /- 1.7 percent (normal 6.0 /- 2.0 percent; p less than 0.001). The reticulocyte count, available in four cases, was 0.23 /- 0.14 and correlated negatively with the GHb. The average GHb in the HS family was 3.9 /- 0.8 percent, which was significantly less than the normal of 6.0 /- 2.0 percent (p less than 0.001); the HbF was less than 1.0 percent. It is concluded that the GHb is diminished in hemolytic anemias not associated with hemoglobinopathies and that this lowering reflects the shortened red cell life span in these processes. To our knowledge, this is the first report of low GHb in hemolytic anemia not associated with hemoglobinopathy, by the affinity chromatographic technique, as opposed to the cation-exchange chromatographic technique.
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5/17. Acute non-immune haemolytic anaemia during infectious mononucleosis unmasking spherocytosis.

    We report a case of acute haemolytic anaemia complicating infectious mononucleosis in a 19-year old male. There was no evidence for an immune haemolysis and red cell studies revealed a previously undiagnosed congenital spherocytosis. We discuss this rare presentation.
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6/17. Inhibition of erythropoiesis by human parvovirus-containing serum from a patient with hereditary spherocytosis in aplastic crisis.

    Aplastic phase serum from a patient with aplastic crisis of hereditary spherocytosis, which was demonstrated to contain human parvovirus, inhibited in vitro erythroid colony formation almost completely. Human parvovirus was resistant to heating for 30 min at 56 degrees C. The suppressive effect of the serum was completely abrogated by adding convalescent phase serum from another patient with aplastic crisis of hereditary spherocytosis. Some normal sera had similar neutralizing ability. The results suggested that aplastic crisis of a patient with hereditary spherocytosis is caused by human parvovirus and that the neutralizing test could offer a tool for predicting the future occurrence of aplastic crisis in the patients with chronic hemolytic anemia.
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ranking = 3.5
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7/17. Human parvovirus-associated red cell aplasia in the absence of underlying hemolytic anemia.

    Human parvovirus (HPV) infection has recently been implicated as the cause of aplastic crisis in patients with hemolytic anemias such as congenital spherocytosis and sickle cell anemia. The virus causes a transient red cell aplasia which, in patients with a shortened red cell life span, is manifested as a rapid worsening of the anemia and an absence of peripheral reticulocytosis. Recovery is associated with the presence of giant pronormoblasts in the bone marrow, and several days later, a brisk peripheral reticulocytosis. In normal subjects, HPV causes erythema infectiosum (fifth disease) but is not associated with symptomatic anemia, probably because of the duration of the normal red blood cell life span. A case of HPV infection producing severe anemia in an immunocompromised patient without an underlying hemolytic anemia is presented here. infection in this patient, a 3-year-old boy with acute lymphoblastic leukemia in remission, may have been prolonged by immunosuppression, leading over a 4-week period to a severe anemia. The immunosuppressed appear to be another group of patients at risk of developing symptomatic anemia when infected by HPV.
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8/17. Glucuronyl transferase deficiency and mild hereditary spherocytosis: effect of splenectomy.

    In a 6-year-old girl an association of hereditary spherocytosis and a defect in hepatic bilirubin metabolism has been found. The patient suffered from mild compensated haemolytic anaemia and excessive hyperbilirubinaemia (maximum concentration 581 mumol/l), the serum activity of liver enzymes was slightly increased. Examination of the erythrocyte membrane proteins revealed a deficiency of the major membrane skeletal protein, spectrin (about 75% of normal) which is probably the basic genetic defect of hereditary spherocytosis. Examination of the patient's family revealed a recessive mode of inheritance. The concentration of bilirubin conjugates in the patient's serum was decreased due to a reduced UDP-glucuronyl transferase activity found in homogenates of liver tissue. Histological liver examination showed an intrahepatic cholestasis, which is a secondary and reversible alteration resulting from severe hyperbilirubinaemia. After splenectomy, normalization of the increased haemolysis and hepatic dysfunction was observed. The excessive hyperbilirubinaemia can be explained by the association of an increased bilirubin load due to haemolytic anaemia and the diminished hepatic conjugation of bilirubin.
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ranking = 3
keywords = spherocytosis
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9/17. 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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keywords = spherocytosis
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10/17. Hereditary spherocytosis presenting in pregnancy.

    A family with hereditary spherocytosis is described in which 3 sisters had haemolytic episodes during six pregnancies. None was treated with splenectomy and each pregnancy has resulted in the birth of mature live infants. Other members of the family were asymptomatic. When not pregnant, the 3 sisters were also asymptomatic. The spectrum of clinical and haematological features in hereditary spherocytosis is discussed. In patients presenting with hereditary spherocytosis and haemolysis during pregnancy, the necessity for splenectomy is assessed.
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ranking = 3.5
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