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Severe microcytosis in a hemoglobin E/Β-thalassemia patient with signs of iron deficiency: A case report

BACKGROUND: β-thalassemia is a hereditary disorder characterized by a decrease in the synthesis of β-globin chains that decreases hemoglobin in erythrocytes, low erythrocyte production, and anemia. CASE PRESENTATION: A 6-year-old girl came with complaints of paleness for one week. Physical examinati...

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Detalles Bibliográficos
Autores principales: Goretti, Laurensia, Adiatmaja, Christophorus Oetama, Kahar, Hartono
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9207008/
https://www.ncbi.nlm.nih.gov/pubmed/35734663
http://dx.doi.org/10.1016/j.amsu.2022.103826
Descripción
Sumario:BACKGROUND: β-thalassemia is a hereditary disorder characterized by a decrease in the synthesis of β-globin chains that decreases hemoglobin in erythrocytes, low erythrocyte production, and anemia. CASE PRESENTATION: A 6-year-old girl came with complaints of paleness for one week. Physical examination showed vital signs within normal limits, conjunctival anemia, and hepatomegaly. Investigations: HGB 5.4 g/dL, MCV 44.5 fL, MCH 15.5 pg, MCHC 34.8 g/dL, RDW-CV 29.2%, WBC 4,770/μL, PLT 2,728,000/μL, Serum iron 29 g/dL, TIBC 217 g/dL and transferrin saturation of 13.36%. Peripheral blood smears showed target cells, teardrop cells, ovalocytes, fragmentocytes, cigar cells, and pseudothrombocytosis by automated hematology tools caused by the misinterpretation of small erythrocytes as platelets. Hemoglobin electrophoresis showed a decrease in HbA (4.9%), as well as an increase in HbF (18.3%), HbE (70.5%), and HbA2 (6.3%). The patient was diagnosed with β-thalassemia. DISCUSSION: Thalassemia with severe microcytosis suggests possible coexistence with iron deficiency. A complete iron profile examination is required in these patients to ensure appropriate and comprehensive medical management. CONCLUSION: Iron profile examination plays an essential role in the management and diagnosis of β-thalassemia patients.