To the editor: Polycythemia is a rare disorder in children and the vast majority of cases are secondary to causes such as cyanotic congenital heart disease, congenital lung diseases, liver and kidney disease, and masses producing erythropoietin. Mutations in exon 3 of the VHL gene are the most common cause of congenital erythrocytosis [1–3]. Several reports have shown evidence of heterozygous a...

Recent studies have shown decreased megakaryocyte expression of the thrombopoietin receptor (c-mpl) in patients with polycythemia vera (PV) but not in those with reactive erythrocytosis. We examined the diagnostic utility of this observation in 22 patients with PV, 7 patients with secondary erythrocytosis (SE), and 10 normal controls. Commercial antibodies against c-mpl were used with standard ...

Recent immigrants from Southeast Asia were screened for hematologic abnormalities using a multichannel cell counter (Coulter S), peripheral smear, free erythrocyte protoporphyrin (FEP), isoelectric focusing, and a qualitative screen for glucose-6-phosphate dehydrogenase deficiency. Hematologic abnormalities were further defined by hemoglobin electrophoresis, globin electrophoresis, HbA2 levels,...

for screening for Hb variants, such as electrophoresis, IEF, or ion-exchange HPLC, are not sufficient because 33 of the 89 different Hb variants associated with erythrocytosis do not induce any major change in net charge of the Hb molecule. The additional use of ESMS is encouraged because combination of this technique and IEF will increase the sensitivity for Hb variants that cause erythrocytos...

Five patients, four women and one man, age 32-8- yr, all whites, had refractory anemia with the same abnormal bone marrow karyotype, i.e., a partial deletion of the long arm of the No. 5 chromosome. The hematologic syndrome was practically the same in these five cases. Examination of the blood revealed a moderate to severe, generally macrocytic anemia with slight leukopenia but normal or elevat...

Background and Purpose: Primary hematologic abnormalities are a rare but established cause of ischemic stroke. In addition, activation of hemostatic parameters is often present during the acute phase of stroke. However, it is uncertain whether these abnormalities occur in both cortical and lacunar infarction; this study aimed to further assess this issue. Methods: Hematologic parameters (prothr...

Abnormalities of hemoglobin synthesis are usually inherited but may also arise as a secondary manifestation of another disease, most commonly hematologic neoplasia. Acquired hemoglobin disorders can be seen in any population and are not restricted to areas of the world with high incidences of inherited hemoglobinopathies. In fact, the acquired hemoglobinopathies may be more readily recognized w...