Laminin B receptor gene (LBR) is located on chromosome 1q41-43. Huet reported another case of hyposegmented granulocytes in 1932. Pelger observed the first case of hyposegmented granulocytes in 1928.
PELGER HUET ANOMALY HOW TO
How to cite this URL: Padmapriya B, Kartheek B V, Bodhireddy SR, Atla B. How to cite this article: Padmapriya B, Kartheek B V, Bodhireddy SR, Atla B. Keywords: Benign, familial, hyposegmented granulocytes, Pelger-Huet anomaly Further work up in family members revealed her siblings and her son were also having Pelger-Huet neutrophils in peripheral smear. Here, we report a case of PHA in neutrophils of a 46-year-old female found during routine peripheral smear examination. Knowledge about PHA is necessary to avoid misdiagnosis of hyposegmented granulocytes as increased band forms (bandemia) and to differentiate it from pseudo Pelger-Huet cells found in certain pathological conditions. Granulocytes have hyposegmented nuclei with normal cytoplasmic granularity and perform immune function. Pelger-Huet anomaly (PHA) is a rare benign genetic disorder affecting terminal differentiation of granulocytes. In pseudo-Pelger-Huët anomaly, cells may appear morphologically similar to inherited Pelger-Huët anomaly, but absence of these findings in other family members, a low percentage of affected cells (usually 5-20%), and involvement of other cell lines (eg, anemia or thrombocytopenia) suggest an acquired anomaly.DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_23_20 Abstract When Pelger-Huët cells are identified, initially attempt to determine if the patient has a benign inherited anomaly or an acquired morphologic feature (ie, pseudo–Pelger-Huët anomaly). Usually, the congenital form is not associated with thrombocytopenia and leukopenia, so if these features are present more detailed search for myelodysplasia is warranted, as pseudo-Pelger–Huët anomaly can be an early feature of myelodysplasia. Pseudo Pelger-Huët anomaly and micromegakaryocytes are considered to be highly characteristic and highly pathognomonic of MDS. The acquired pseudo Pelger-Huët anomaly has been associated with pathologic conditions including acute and chronic myeloid leukemias, myelodysplastic syndrome (MDS), severe infections and some other toxic conditions. The morphologic characteristic seen in pseudo Pelger-Huët anomaly is similar to Pelger-Huët anomaly. In the inherited anomaly, affected neutrophils with bilobed nuclei make up 60-90% of the neutrophils seen.Īnomalies resembling Pelger-Huët anomaly that are acquired rather than familial have been described as pseudo Pelger-Huët anomaly. Neutrophils contain not more than two lobes to the nuclei (spectacle-shaped nuclei) and “band forms” are frequent.
In 1931 Huet, a Dutch pediatrician, identified it as an inherited disorder. The characteristic leukocyte appearance was first reported in 1928 by Pelger, a Dutch hematologist, who described leukocytes with dumbbell-shaped bilobed nuclei, a reduced number of nuclear segments, and coarse clumping of the nuclear chromatin.
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