• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.17 no.4
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• pp.266-269
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• 2014

Gilbert syndrome is the most common inherited disorder of bilirubin glucuronidation. It is characterized by intermittent episodes of jaundice in the absence of hepatocellular disease or hemolysis. Hereditary spherocytosis is the most common inherited hemolytic anemia and is characterized by spherical, osmotically fragile erythrocytes that are selectively trapped by the spleen. The patients have variable degrees of anemia, jaundice, and splenomegaly. Hereditary spherocytosis usually leads to mild-to-moderate elevation of serum bilirubin levels. Severe hyperbilirubinemia compared with the degree of hemolysis should be lead to suspicion of additional clinical conditions such as Gilbert syndrome or thalassemia. We present the case of a 12-year-old boy with extreme jaundice and nausea. The diagnosis of hereditary spherocytosis was confirmed by osmotic fragility test results and that of Gilbert syndrome by genetic analysis findings.

• Pediatric Infection and Vaccine
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• v.16 no.2
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• pp.215-219
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• 2009

Mycoplasma pneumoniae is a common cause of community-acquired pneumonia in children, with a peak incidence at 5-14 years. Extrapulmonary manifestations occur in 20-25% of patients with M. pneumoniae infection. Most auto-antibodies that cause immune hemolytic anemia in humans are cold agglutinins. The formation of cold agglutinins is frequently observed during M. pneumoniae infections, and cold agglutinin disease usually occurs during M. pneumoniae infections. Nevertheless, severe hemolysis is exceptional. If a patient has any underlying disease related to hemolysis, it is possible to accelerate hemolysis. Hereditary spherocytosis is a common cause of hereditary hemolytic anemia resulting from red blood cell membrane defects. Hemolysis of red cells may result from corpuscular abnormalities or extracorpuscular abnormalities, such as immune or non-immune mechanisms. We report a case of hereditary spherocytosis associated with severe hemolytic anemia due to Mycoplasma pneumonia.

• Journal of Chest Surgery
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• v.46 no.2
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• pp.156-158
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• 2013

Thoracic extramedullary hematopoiesis (EMH) is a rare disease entity that is usually associated with hematologic disorders, such as myelodysplastic or hemolytic disease. Because thoracic EMH is usually encountered as a mass during radiologic examinations, it should be differentiated from posterior mediastinal neurogenic tumors. Here, the authors report a case of EMH associated with hereditary spherocytosis. The patient underwent a complete excision by thoracoscopic surgery to differentiate it from other mediastinal tumors.

• Journal of Yeungnam Medical Science
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• v.11 no.1
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• pp.42-48
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• 1994

Among the erythrocyte membrane defects, hereditary spherocytosis is the most common. The erythrocyte membrane defect results from a deficiency of spectrin, the most important structural protein in red cell. Hereditary spherocytosis often presents with hemolytic anemia, jaundice, moderate splenomegaly. Diagnosis is established by the presence of spherocytes in the peripheral blood, reticulocytosis, an increased osmotic fragility, and a negative Coombs test. In children, splenectomy is usually performed after age 6 years but can be done at a younger age if warranted by the severity of the anemia and the need for frequent transfusions. In the period December 1987 to Agust 1993, 9 patients with hereditary spherocytosis underwent splenectomy and the following results were obtained. 1. Nine patients were comprised of five males and four females. 2. Five patients(55.6%) had been admitted to our hospital during age 6-10 years. 3. Four of the nine patients had autosomal dominant inheritance with variable expression. The other five patients had no known inheritance. 4. The diagnosis of the spherocytosis was based on the increased osmotic fragility and increased autohemolysis of the erythrocytes, as well as on the appearance of spherocytes in the peripheral blood smear. 5. In all cases splenectomy was performed. Two patients had concomitant gall stones and choledocholithiasis, respectively. One patient with concomitant gall stones underwent simultaneous cholecystectomy and splenectomy. The other patient associated with choledocholithiasis underwent splenectomy, cholecystectomy, choledocholithotomy, and T-tube drainage. 6. Complete hematologic recovery was obtained by the splenectomy in all cases. 7. Postoperative complication was not occurred.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1139-1142
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• 2003

Human parvovirus(HPV) B19 infection causes erythema infectiosum in children, sometimes red cell aplastic crisis with hemolytic anemia and chronic bone marrow failure in immunocompromised hosts. HPV B19 is directly cytotoxic for erythroid progenitor cells and inhibits erythropoiesis. Infrequently, HPV B19 inhibits hematopoiesis of three cell lineages and causes transient pancytopenia in patients with hemolytic disorders. We report three patients with hereditary spherocytosis who developed transient aplastic crisis. A HPV B19 infection was confirmed by IgM anti-B19 parvovirus titers and characteristic findings of bone marrow examination as the causative agent associated with severe pancytopenia. Three patients recovered spontaneously after a short period of supportive care with red cell transfusions and intravenous immunoglobulin.

• Advances in pediatric surgery
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• v.3 no.2
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• pp.160-163
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• 1997

Three cases of laparoscopic splenectomy for hereditary spherocytosis are reported. The average operation time was 100 minutes. This was longer than traditional open splenectomy for the same entity(63 minutes). Average hospitalization period was 3 days. This was shorter than the hospitalization period for the traditional group(6.2 days).

• Korean Journal of Veterinary Research
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• v.52 no.3
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• pp.209-211
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• 2012

A 5-year-old castrated male Shih-tzu dog and a 9-year-old intact female Schnauzer dog were presented with anorexia and depression. These 2 dogs were diagnosed based on which criteria as primary immune-mediated hemolytic anemia (IMHA). Blood examination showed anemia, spherocytosis, auto-agglutination and total bilirubinemia in 2 cases. These dogs were recovered by treatment of mycophenolate mofetil and prednisolone, and showed good prognosis until now. Mycophenolate mofetil is recommended as initial treatment in canine IMHA.

• Journal of Veterinary Clinics
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• v.13 no.1
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• pp.96-101
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• 1996

면역성 용혈성 빈혈증 병견은 임상적으로 허약, 황달, 발열, 침울 및 점막창백을 나타낸다. 본 병의 진단은 구상적혈구증가증, 혈구응집반응 또는 직접적 Coomb시험 양성반응을 확인함과 동시에 용혈성 빈혈의 다른 원인을 배제함으로서 확정하여야 한다. 치료방법에는 적절한 보조적 요법과 함께 면역억압제가 포함된다. 일반적으로 치료에 있어서는 일차적으로 glucocorticoids가 선택되는데 흔히 cytoxan, aziothioprin, vincristine 또는 danazole과 같은 다른 약제와 병용된다. 치료는 그 반응에 근거하여 면역억압제의 용량을 2-4주 간격으로 점차 감량하면서 6개월 또는 그 이상까지 계속한다. 면역억압제에 대한 치료반응은 지연될 수 있고 또 적혈구 보유가 골수로부터 새로운 적혈구를 유리하게될 때까지 병축을 유지시키기에 부적당할 수 있기 때문에 병축의 예후판정은 경계하여야 한다. 심급성 내지 급성 예는 일반적으로 예후가 보다더 좋지 않다.

• Korean Journal of Veterinary Research
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• v.63 no.3
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• pp.28.1-28.5
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• 2023

A 10-year-old spayed female Maltese presented with purpura and hematemesis. Initial laboratory evaluation revealed immune-mediated thrombocytopenia, but evidence of hemolytic anemia was not identified. Three milligrams of human intravenous immunoglobulin (hIVIG) was administered for 3 hours following prednisolone and mycophenolate mofetil. A pale mucous membrane was identified, and the packed cell volume decreased by 3%. Blood film examination revealed significant spherocytosis with auto-agglutination. Blood transfusions and immunosuppression were continued for 4 days, and hIVIG was discontinued. This report describes a case of increased immune-mediated hemolysis after hIVIG administration, possibly due to new-onset immune-mediated hemolytic anemia or enhanced immunogenicity.

• Clinical and Experimental Pediatrics
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• v.50 no.6
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• pp.511-518
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• 2007

To understand the hemolytic anemia (HA) in children, the diagnostic approach and management of hereditary and acquired HA are described. The hereditary hemolytic anemia (HHA) can be classified according to the pathogenesis into three types : RBC membrane defects, hemoglobinopathies, and RBC enzymopathies. Clinical characteristics, laboratory findings and molecular defects of these three types are presented briefly. In Korea, HHA due to the RBC membrane defect, hereditary spherocytosis had been reported often but HHA due to hemoglobinopathies and RBC enzymopathies had been thought to be relatively rare. With recent development in the molecular diagnosis, ${\beta}$ thalassemia, mostly heterozygote, G6PD and pyruvate kinase deficiency have been reported with gene characterization. If the patients with microcytic hypochromic anemia show unproportionally low MCV or MCH or refractory to the iron therapy, hemoglobin electrophoresis and gene analysis for thalassemia or other unstable hemoglobinopathies need to be done accordingly. The global movement of the population especially from the region prevalent of hemoglobinopathies or enzymopathies to Korea warrants considering broad spectrum of etiology for the diagnosis of HHA. Aquired HA resulting from extracellular factors such as autoimmune HA from warm antibody, cold agglutinin and paroxysmal cold hemoglobinuria as well as nonimmune HA are described briefly.