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The lymphoreticular system and bone marrow
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
In this condition there is severe hypofunction of the bone marrow. It is most often acquired and may follow exposure to drugs (e.g. chloramphenicol, gold, indometacin) or chemicals (e.g. benzene, insecticides) and viral infections such as hepatitis, CMV, and parvoviruses. In almost half the cases no cause is found (idiopathic), although an autoimmune attack at the level of the stem cell is considered likely because patients often respond to immunomodulatory drugs. Congenital aplastic anaemia occurs as part of Fanconi's anaemia. Rarely, aplasia may be confined to the red cell series – this is termed ‘pure red cell aplasia’. It may be congenital or acquired, especially secondary to thymoma.
Chronic Leukemias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Scott J. Graham, James D. Cotelingam
Examination of the peripheral blood reveals varying numbers of large lymphocytes with irregular nuclei and abundant pale cytoplasm containing azurophilic granules of variable size and number (Fig. 8). Absolute LGL counts range from 2.0 to 7.0 × 109/L. The accompanying neutropenia, if present, may be cyclical. Slight anemia and thrombocytopenia is often observed. Pure red cell aplasia has occasionally been reported.
Myelodysplastic Syndromes
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Pure red cell aplasia is a rare disease that can be congenital or acquired. The congenital form, called Diamond–Blackfan anemia, seen in childhood is associated with physical abnormalities, such as craniofacial abnormalities and renal or cardiac defects. The acquired chronic form of pure red cell aplasia can be primary or secondary to various factors, including infections, malignancies, collagen vascular disease, or drugs. Large granular lymphocytes (T-LGL) leukemia is the most common underlying hematological malignancy (an associated thymoma is found in up to 22% of patients). The BM is pure red cell aplasia and either hypocellular or normocellular, and typically shows selective decrease or absence of red cell precursors beyond the proerythroblast stage (the maturation of the granulocytic and megakaryocytic series is normal). Iron storage is often increased (due to blood transfusions). There is frequently an increase in hematogones. In parvovirus infections, enlarged proerythroblasts with intranuclear inclusions may be seen and the dyserythropoiesis might very prominent.
Low- and intermediate-risk myelodysplastic syndrome with pure red cell aplasia
Published in Hematology, 2021
Huaquan Wang, Haiyue Niu, Tian Zhang, Limin Xing, Zonghong Shao, Rong Fu
Pure red cell aplasia (PRCA) is a rare bone marrow failure syndrome only involving erythrocytes. Its clinical features include severe anemia, reduced reticulocytes, absence or severe reduction of bone marrow erythroid precursors, and normal white blood cells and platelets [1]. Myelodysplastic syndrome (MDS) is a group of heterogeneous malignant clonal hematopoietic stem cell diseases, with dysplasia in one or multiple lineage of myeloid blood cells, ineffective hematopoiesis, and high risk of transformation to acute myeloid leukemia (AML) [2]. Erythroid dysplasia is one of the distinctive features of MDS. Absence or extreme reduction of erythroid precursors is a rare form of erythroid dysplasia. A very small number of patients with MDS onset in the form of PRCA [3]. The differential diagnosis of low- and intermediate-risk MDS and PRCA is very difficult, especially for MDS without blasts.
Caution the masker of Good’s sydrome on the secondary pure red cell aplasia
Published in Hematology, 2021
Weiwei Chen, Lei Jiang, Jinlin Liu
We read the Rong’s paper [1] with great interest. In this article, a total of 53 acquired pure red cell aplasia (PRCA) patients, including 30 idiopathic PRCA and 23 secondary PRCA patients, were retrospectively analyzed. Their data was determined that thymoma and viral infections were the most common causes for secondary PRCA. However, in this article, the results of IgG level or CD19+B cells number in the peripheral blood were not examined, although the CD5+CD19+ activated B cells were investigated in 46 PRCA patients.
The clinical characteristics and therapy response of patients with acquired pure red cell aplasia
Published in Hematology, 2018
Rong Fu, Tian Zhang, Bingnan Liu, Jia Song, Guojin Wang, Lijuan Li, Huaquan Wang, Limin Xing, Yuhong Wu, Jing Guan, Zonghong Shao
Pure red cell aplasia (PRCA) is a rare hematopoietic disorder characterized by normocytic and normochromic anemia, reticulocytopenia and absence of erythroid precursors in bone marrow. The clinical characteristics of patients with acquired PRCA in China were poorly understood at present. The clinical features, immune state and treatment response of acquired PRCA patients diagnosed in our hospital from January 2007 to January 2017 were retrospectively analyzed in this study. The results showed that thymoma (13.21%) and parvovirus B19 infection (11.32%) were the most common causes for secondary PRCA. Ferritin (Fer) levels (784.02 ± 643.03 ng/mL) and erythropoietin (EPO) levels (10 of 34 patients were 20–400U/L, 20 of 34 patients were >400 U/L) were increased in PRCA patients. The total CR and PR rate of immunosuppressive therapy (IST) were 68.29% and 12.20%, respectively. Patients with EPO level >400 U/L and Fer level >200 ng/ml had significantly lower CR rate than others (57.14% vs. 88.98%, and 57.10% vs. 91.67%, respectively). The patients with EPO level >400 U/L also had longer hemoglobin recovery time (45.57 ± 12.75 days). Patients treated with corticosteroids (CS) + cyclosporine A (CsA) had lower relapse rate compared to the CS group (29.17% vs. 80.00%). In conclusion, patients with PRCA had high EPO and Fer levels. The immune pathogenesis was heterogeneity in acquired PRCA. Thymoma and viral infections are the most common causes for secondary PRCA. Secondary PRCA has more abnormal immune and longer recovery time than idiopathic PRCA. IST is effective for both idiopathic and acquired PRCA, but with high relapse rate. Therapy of CS + CsA could reduce the relapse rate of PRCA. Increased EPO and Fer levels might be the negative factors for prognosis of acquired PRCA. The most common complication of acquired PRCA is infection during treatments.