High‐oxygen‐affinity hemoglobinopathy‐associated erythrocytosis: Clinical outcomes and impact of therapy in 41 cases
| Autor: | James D. Hoyer, Naseema Gangat, Mrinal M. Patnaik, Ayalew Tefferi, Jennifer L. Oliveira, Animesh Pardanani |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Adult
Male Serum erythropoietin medicine.medical_specialty Polycythemia Hematocrit Gastroenterology Young Adult Phlebotomy High oxygen hemic and lymphatic diseases Internal medicine Humans Medicine Family history Child Aged Retrospective Studies Aged 80 and over medicine.diagnostic_test business.industry Disease Management Infant Hematology Middle Aged medicine.disease Thrombosis Hemoglobinopathies Oxygen Hemoglobinopathy Female Hemoglobin business Platelet Aggregation Inhibitors |
| Zdroj: | American Journal of Hematology. 96:1647-1654 |
| ISSN: | 1096-8652 0361-8609 |
| DOI: | 10.1002/ajh.26375 |
| Popis: | We describe presenting features, treatment strategies, and follow-up events involving 41 patients (median age 39 years, range 1-81; 54% males) with high oxygen affinity (HOA) hemoglobinopathy-associated erythrocytosis, seen at our institution (1973-2020). Thirty-four (83%) patients carried β-chain (13 Malmo, 4 Olympia, 3 San Diego, 2 Wood) and 7 (17%) α-chain (4 Dallas and one each Columbia-Missouri, Jackson, and Wayne) variants. Median (range) hemoglobin (Hgb)/hematocrit (Hct), serum erythropoietin and p50 were 18 g/dL/52.9% (16-21.9/48-66), 10.4 mIU (4-36.3), and 20 mmHg (12-25), respectively. Family history was documented in 24 patients and history of thrombosis in two (5%). Treatment included phlebotomy in 23 and antiplatelet therapy in 21 patients. At a median follow-up of 10 years, 23 (56%) patients reported one or more symptoms that were thought to be related to their increased Hct while thrombosis was documented in 10 (24%) patients. Neither Hgb/Hct level nor active phlebotomy showed a significant correlation with either thrombotic or nonthrombotic symptoms (p > .1 in all instances). Among 23 pregnancies recorded, 78% resulted in live births and no fetal loss was attributed to erythrocytosis. The current study does not implicate Hgb/Hct level as a major contributor of morbidity in HOA hemoglobinopathy-associated erythrocytosis and suggests limited therapeutic value for phlebotomy. |
| Databáze: | OpenAIRE |
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