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[2] Milder or atypical cases, however, are at times difficult to diagnose.[1, 2] Some membrane protein defects in HS are identified using sodium dodecylsulfate�Cpolyacrylamide gel electrophoresis (SDS-PAGE).[1-3] Furthermore, the diagnostic value of the eosin-5-maleimide (EMA) binding test has been validated in some studies with understanding of its limitations.[2-4] Nevertheless, the predictive value of these assays with regard to the clinical severity of aplastic crisis has been poorly studied and remains controversial.[5] To our knowledge, this is the first report of a familial case that resulted in different Histone demethylase aplastic crisis outcomes for two patients with HS, despite similar results using both EMA binding test and SDS-PAGE. Aplastic crisis was defined as a drop of hemoglobin of at least 2?g/dL from baseline with reticulocytopenia (reticulocyte count Selleckchem Venetoclax phototherapy, and erythroresistant test results indicated that the erythrocytes had mild osmotic fragility. Many spherocytes were present on scanning electron microscopy (SEM). After infancy, she was asymptomatic, with the exception of a slightly enlarged spleen (1?cm below the left costal margin). The patient had neither gallstones nor required blood transfusion. The specific protein abnormality of RBC was established using SDS-PAGE and densitometric this website quantification.[3, 5] The results showed that protein 4.2 was slightly reduced. The fluorescence intensity of the EMA-labeled RBC was quantified using flow cytometry,[3] and indicated decreased fluorescence intensity. Results of assays 1?year before admission are given in Table?1. The girl had been diagnosed with mild HS, according to the previously defined criteria.[1, 2] On 5 July 2011, the patient was admitted with suspected aplastic crisis. Ten?days before admission she made contact with a susceptible friend with a parvovirus B19 infection. The patient became febrile 2?days before admission. On examination, the patient had a fever, and the spleen was palpable 2?cm below the left costal margin. The blood pressure was 112/65?mmHg and the pulse was 88?beats/min. She did not complain of any pain in the abdomen or legs. The blood count was as follows: white blood cell (WBC) count, 4.59 �� 109?cells/L; hemoglobin (Hb), 10.3?g/dL; platelet count, 227 �� 109/L. The serum bilirubin concentration was 1.4?mg/dL. As shown in Figure?1, the patient's fever persisted for 3?days, and erythema appeared temporarily on her cheek and extremities. WBC count and platelet count increased after admission, and maximum lactate dehydrogenase (LDH) was 368?IU/L.