PMID- 9202619 OWN - NLM STAT- MEDLINE DCOM- 19970717 LR - 20220317 IS - 0022-3476 (Print) IS - 0022-3476 (Linking) VI - 130 IP - 6 DP - 1997 Jun TI - Screening for hereditary spherocytosis by use of automated erythrocyte indexes. PG - 957-60 AB - OBJECTIVE: To determine whether the mean corpuscular hemoglobin concentration (MCHC) or other erythrocyte indexes, as determined by automated cell counters, remains a useful screening test for identifying patients with hereditary spherocytosis (HS). METHODS: Erythrocyte indexes from 112 children with HS who had not undergone splenectomy were compared with those measured in an equal number of healthy, age-matched children. All indexes were derived from measurements obtained by aperture impedance. RESULTS: Mean corpuscular hemoglobin concentration in the HS group was 35.9 gm/dl, significantly higher than in normal control subjects (34.3 gm/dl; p < 0.001). Mean erythrocyte distribution width also was significantly higher in patients with HS (19.3 vs 12.6; p < 0.001). The MCHC distinguishes individuals with HS, with an area under the receiver operating characteristic curve of 0.86. Although not disease specific, an erythrocyte distribution width > 14 has 85% sensitivity and 97% specificity and an area under the receiver operating characteristic curve of 0.92. An MCHC > 35 gm/dl has a sensitivity of 70% and a specificity of 86%. Combining the MCHC and erythrocyte distribution width increases the area under the receiver operating characteristic curve to 0.97. Specificity is 100% and likelihood ratio is infinite when both the MCHC and erythrocyte distribution width are elevated. CONCLUSIONS: The automated MCHC is an effective screening test to identify children with HS. An elevated erythrocyte distribution width adds additional specificity and is itself a powerful screening tool. The combination of the two tests is an excellent predictor for the diagnosis of HS. FAU - Michaels, L A AU - Michaels LA AD - Division of Hematology, Children's Hospital of Philadelphia, Pennsylvania 19104, USA. FAU - Cohen, A R AU - Cohen AR FAU - Zhao, H AU - Zhao H FAU - Raphael, R I AU - Raphael RI FAU - Manno, C S AU - Manno CS LA - eng PT - Comparative Study PT - Journal Article PL - United States TA - J Pediatr JT - The Journal of pediatrics JID - 0375410 SB - IM MH - Adolescent MH - Adult MH - Child MH - Child, Preschool MH - *Erythrocyte Indices MH - Humans MH - Infant MH - Reticulocyte Count MH - Spherocytosis, Hereditary/*diagnosis MH - Splenectomy EDAT- 1997/06/01 00:00 MHDA- 1997/06/01 00:01 CRDT- 1997/06/01 00:00 PHST- 1997/06/01 00:00 [pubmed] PHST- 1997/06/01 00:01 [medline] PHST- 1997/06/01 00:00 [entrez] AID - S0022-3476(97)70283-X [pii] AID - 10.1016/s0022-3476(97)70283-x [doi] PST - ppublish SO - J Pediatr. 1997 Jun;130(6):957-60. doi: 10.1016/s0022-3476(97)70283-x.