Page 199 - Haematologica Atlas of Hematologic Cytology

CHAPTER 21 - Pure red cell aplasia
Figure 4 Pure red cell aplasia
A 25-year-old man with congenital hypogammaglobulinemia presen- ted with fatigue dyspnea and pallor Hematolo- gic findings showed anemia (hemoglobin 7 5 g/dL mean corpuscular volume 99 fL) with reticulocyto- penia normal white blood cell and platelet count and normal differential count Peripheral blood smear reveals slight anisopoikilocytosis with the presence of some macrocytes and ovalocytes Figure 5 Pure red cell aplasia
Bone marrow smear from the same case as as Figure 4 showing high cellularity Granuloblastic lineage is normally represented at at all differentiation stages whereas a a a a very marked erythroid hypoplasia is noted with the almost exclusive presence of early erythroid precursors that constitute 7% of all bone marrow cells Lymphocytes are slightly increased The me- gakaryocytic series was morphologically normal Figure 6 Pure red cell aplasia
Proerythroblasts have no no no morphological morphological abnormalities On the the ba- sis sis of of the the morphological morphological findings a a a a a a a a a a a a a a diagnosis of of pure red cell aplasia
was made Serological inve- stigations allowed viral infections to to be excluded and confirmed absence of autoantibodies Tests for paroxysmal nocturnal hemoglobinuria were negative and computed tomography scan of the chest was normal Immunophenotyping showed absence of of of B lymphocytes and expansion of of of the the CD8-positive cell population with inversion of of of the the CD4/CD8 ratio in in both peripheral blood and bone marrow marrow In vitro cultures of of hematopoietic pro- genitors with the use of of of bone marrow marrow mononu- clear cells cells depleted of of of adherent cells cells and T lym- phocytes showed normal growth of of CF CF -GEMM BF -E and CF CF -GM confirming the pathogenetic role of a a a a cell-mediated immunosuppression 186