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Clinical Characteristics of Pediatric Patients with Congenital Erythrocytosis: A Single-Center Study
AbstractAlthough congenital erythrocytosis (CE), an inherited disorder, impairs pediatric quality of life, physicians often overlook high hemoglobin (Hgb) levels and its symptoms due to lack of knowledge of age-adjusted pediatric Hgb levels and CE ’s rarity. In a retrospective, single-center study, data from hospital records of pediatric patients diagnosed with CE were evaluated. Twenty-six patients from 25 families (80.8% male) had been diagnosed with CE in 20 years, at a mean age of 14.9 ± 2.8 years (8.3–17.8) and with a mean Hgb level of 17.36 ± 1.44 g/dL (14.63–22.1). No serum erythropoietin levels e...
Source: Indian Journal of Hematology and Blood Transfusion - August 25, 2021 Category: Hematology Source Type: research

Recurrent right middle cerebral artery territory infarctions in a patient with JAK2 (V617F) point mutation positive polycythemia rubra vera (P2.258)
CONCLUSIONS: Hyperviscosity in polycythemia with hematocrit of more than 43[percnt] is known to cause ischemic events and it is evident in our case where our patient had recurrent ischemic strokes whenever the HCT is about 50[percnt] and the right MCA M1 segment though a mild stenosis, it becomes a critical factor for this patient as the patient had recurrent right MCA territory infarcts and hence in addition to regular phlebotomy to keep hematocrit less than 43[percnt], our patient might also need intervention in the form of cerebral angioplasty or stenting to treat even mild arterial stenosis. Study Supported by: ...
Source: Neurology - April 8, 2015 Category: Neurology Authors: Suri Mohanram, S., Nattanmai Chandrasekaran, P. Tags: Cerebrovascular Disease and Interventional Neurology: Genetics and Stroke Source Type: research