Clinico-Hematological Features in Pure Red Cell Aplasia Patients Diagnosed at Diagnostic & Research Laboratory, Hyderabad

Abstract

Pure red-cell aplasia (PRCA) or erythroblastopenia is a rare type of anemia or disorder that can be either idiopathic or associated with certain autoimmune diseases and affect red blood cell (RBC) precursors1,2. The disease is often present at birth, and it manifests in the first year of life in more than 90% of patients. It is a syndrome characterized by normochromic, normocytic anemia, reticulocytopenia (<1%), and an almost complete absence of erythroblasts (<0.5%) from the bone marrow3.

PRCA is categorized into congenital and acquired classes4. Studies elucidating the characteristics of PRCA in Pakistani population are very limited5. The current study was aimed to identify the clinical and hematological features in local PRCA patients.

This cross-sectional study was carried out at Diagnostic & Research Laboratory LUMHS Hyderabad from October 2014 to December 2016. Five patients from Diagnostic & Research Laboratory LUMHS Hyderabad referred for bone marrow biopsy were selected. After taking detailed history and physical examination, clinical and hematological findings were recorded on a pre-designed proforma. Bone marrow aspiration was done on all patients and trephine biopsies were taken only where indicated6. Blood samples were taken and complete blood count (CBC) was performed on XN 1000 five parts fully automated haematology analyzer (Sysmex®, Japan). Peripheral blood films were stained with Leishman Stain and brilliant cresyl blue. These films were screened generally and the reticulocyte count was performed manually.

Patients with normochromic normocytic anemia, low reticulocyte count and isolated erythroblastopenia in the bone marrow were selected6. Whereas those with other types of anemia and patients with abnormal myelopoiesis and/or thrombopoiesis were excluded.