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Year : 2015  |  Volume : 6  |  Issue : 1  |  Page : 39

Hereditary pyropoikilocytosis

Department of Pediatric Hematology, Maternity and Children Hospital, Director of Hereditary Blood Diseases Center, AL Ahsa, Saudi Arabia

Date of Web Publication15-Apr-2015

Correspondence Address:
Muneer Hassan Albagshi
Maternity and Children Hospital, Director of Hereditary Blood Diseases Center, AL Ahsa
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1658-5127.155191

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How to cite this article:
Albagshi MH. Hereditary pyropoikilocytosis. J Appl Hematol 2015;6:39

How to cite this URL:
Albagshi MH. Hereditary pyropoikilocytosis. J Appl Hematol [serial online] 2015 [cited 2023 Jun 4];6:39. Available from: https://www.jahjournal.org/text.asp?2015/6/1/39/155191

A 3-day-old boy neonate referred to the hospital because of indirect hyperbilirubinemia, which was treated with phototherapy.

His complete blood count showed the following: Red blood cell, 5.89 × 10 12 L, Hb, 16.7 g/L; hematocrit, 0.49.91 L/L; mean corpuscular volume, 86.3 fL; mean corpuscular hemoglobin (MCH), 25.9 pg; MCH concentration, 303 g/L; red cell distribution width, 20.8%; platelet, 540 × 10 9 /L; mean platelet volume, 9.1 fL. white blood cell (WBC), 11.55 × 10 9 ; A differential WBC count revealed the following: Neutrophils, 27%, lymphocytes 64%, monocytes 6%, eosinophils 2%, atypical lymphocytes 2%. Both baby and mother have A positive blood group, and direct antiglobulin test was negative. Total bilirubin 25 mg/dl and indirect bilirubin 24 mg/dl.

A morphology assessment of his blood sample peripheral blood with leishman stain [Figure 1] shows marked aniso-poikilocytosis, microcytosis, hypochromia, elliptocytes, and fragmented red cells indicating the diagnosis of hereditary pyropoikilocytosis.
Figure 1: Peripheral blood film

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Membrane disorders are characterized by defective horizontal interactions between membrane proteins. [1],[2],[3] The differential diagnosis is Hereditary elliptocytosis in the severe form.

The patients of such condition need to be followed also for a possible need for red transfusions in the first few years of life.

  References Top

Ogburn PL Jr, Ramin KD, Danilenko-Dixon D, Fairbanks VF, Ramsey PS. In utero erythrocyte transfusion for fetal xerocytosis associated with severe anemia and non-immune hydrops fetalis. Am J Obstet Gynecol 2001;185:238-9.  Back to cited text no. 1
del Giudice EM, Perrotta S, Nobili B, Specchia C, d'Urzo G, Iolascon A. Coinheritance of Gilbert syndrome increases the risk for developing gallstones in patients with hereditary spherocytosis. Blood 1999;94:2259-62.  Back to cited text no. 2
Delhommeau F, Cynober T, Schischmanoff PO, Rohrlich P, Delaunay J, Mohandas N, et al. Natural history of hereditary spherocytosis during the first year of life. Blood 2000;95:393-7.  Back to cited text no. 3


  [Figure 1]


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