Screening for hereditary spherocytosis by use of automated erythrocyte indexes☆,☆☆
Section snippets
Subjects
We reviewed the outpatient medical records of children followed up regularly between 1989 and 1995 at the Children's Hospital of Philadelphia Hematology Clinic for HS. Hereditary spherocytosis was diagnosed on the basis of clinical history, peripheral blood examination, and reticulocyte count, and was confirmed by RBC osmotic fragility. Hemoglobin, hematocrit, MCV, MCHC, RDW, and reticulocyte count were documented from the clinic visits of 128 patients. Data from patients younger than 12
RESULTS
Means and standard deviations of the age, hemoglobin level, RBC indexes, and reticulocyte counts are shown in Table I. Patients with HS who have not undergone splenectomy have a significantly lower mean hemoglobin concentration and a significantly higher MCHC and RDW than normal control subjects.
Hematologic characteristics of patients with HS who had splenectomy and those who did not are also shown in Table I . The group without splenectomy is significantly younger than the splenectomy group.
DISCUSSION
Although no extensive information is available on the use of the automated RBC indexes in the differential diagnosis of microcytic anemias, little attention has been given to their role in the diagnosis of other anemias. In a once active debate over the utility of the automated MCHC in the microcytic anemias, it was assumed that this index was helpful in the diagnosis of HS. 5, 6, 9 However, there is evidence that electronic cell counters underestimate the MCHC with increasing error as the MCHC
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Cited by (59)
Disease-modifying influences of coexistent G6PD-deficiency, Gilbert syndrome and deletional alpha thalassemia in hereditary spherocytosis: A report of three cases
2016, Clinica Chimica ActaCitation Excerpt :Several interesting points may be gleaned about the diagnosis of HS from these cases. A combination of MCHC > 35 g/dl and RDW-CV > 14% was previously reported to be “an excellent predictor for the diagnosis of HS” with 100% specificity and > 85% sensitivity [8]. Only one of our patients, Case 1, would have met this criterion.
Abnormalities of the erythrocyte membrane
2013, Pediatric Clinics of North AmericaCitation Excerpt :The red cell distribution width (RDW) is increased (>14) in most patients. Combining the MCHC and red cell distribution width (>35.4 g/dL and >14, respectively), or combining the MCHC with histograms of hyperdense erythrocytes (MCHC>40 g/dL) obtained from laser-based cell counters, has been utilized to rapidly identify HS patients.21,23 Typical HS patients have blood smears with easily detectable spherocytes (ie, erythrocytes lacking central pallor), which are distinctive but not diagnostic (Fig. 2A).
Clinical Features and Outcome of Children with Hereditary Spherocytosis
2022, Journal of Pediatric Hematology/Oncology
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0022-3476/97/$5.00 + 0 9/21/79371
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Reprint requests: Catherine S. Manno, MD, Division of Hematology, Children's Hospital of Philadelphia, 324 S. 34th Street, Philadelphia, PA 19104.