Pure red cell aplasia (PRCA) is a rare disorder that presents with anemia secondary to the failure of erythropoiesis. It is characterized by normocytic, normochromic anemia, associated with reticulocytopenia in the peripheral blood and absent or infrequent erythroblasts in the bone marrow. It is distinct from aplastic anemia in having intact precursors for platelets and leukocytes, which are normal in number and morphology in the peripheral blood. Kaznelson first described PRCA in 1922. The congenital (or inherited) form of PRCA, also called Diamond-Blackfan syndrome, was first described by Joseph in 1936 and subsequently by Diamond and Blackfan in 1938. The association of PRCA with thymoma led to the discovery of the autoimmune mechanisms involved in the pathogenesis of this rare disease. PRCA has been the object of much laboratory research not only due to the immune mechanisms involved in the destruction of the red blood cells but also because of its association with parvovirus B19 in patients with sickle cell disease. However, due to the rarity of the disease, PRCA can never be evaluated in large controlled clinical trials. As a result, the majority of recommendations have their basis on retrospective trials or anecdotal case reports.
Pure red cell aplasia can either be inherited or acquired.
PRCA is an extremely heterogeneous disease both clinically and pathologically. The pathophysiology is discussed here based on the classification.
Congenital PRCA or Diamond Blackfan anemia (DBA)
Transient Aplastic Crisis and Parvovirus B19 infection
Transient erythroblastopenia of childhood (TEC)
Secondary acquired PRCA
The peripheral smear demonstrates normocytic normochromic anemia with reticulocytopenia. The white cell count and platelet count are normal in number and morphology. The histological picture seen on bone marrow examination depends on the cause of PRCA. Complete absence or near absence of erythroblasts (less than 1% erythroblasts on marrow differential count) from an otherwise normal marrow is a characteristic of autoimmune PRCA. Rarely, a few erythroblasts or basophilic erythroblasts are present, but their number never exceeds 5% of the differential count. In patients with B19 parvovirus infection, large proerythroblasts with vacuolated cytoplasm and pseudopodia (giant pronormoblasts) appear on the marrow exam; however, this is not a diagnostic finding. Lymphoid aggregates along with plasmacytosis and lymphocytes point to an inflammatory reaction. Any signs of hypercellularity, presence of ringed sideroblasts, or dysplastic features that extend beyond one cell line are suggestive of a myelodysplastic variant of PRCA or MDS itself.
There are no specific signs or symptoms associated with PRCA. The most common presentation is the same as that of anemia. Generalized fatigue, decreased exercise tolerance, palpitations, and in extreme cases, presyncope or syncope (when associated with cardiac stress due to increased work of function). The physical exam is also non-specific. Pallor is a feature in all patients. A thorough skin exam is necessary to look for erythema infectiosum, which correlates with parvovirus B19. A prodromal rash is sometimes present in children where a reticular and lacy body eruption is visible. The systemic examination should look for swollen lymph nodes, hepatomegaly, and splenomegaly. None of these signs or symptoms are definitively diagnostic of PRCA by themselves but will provide vital clues in establishing etiology.
Diamond-Blackfan syndrome is associated with physical anomalies in up to a third of all patients. Craniofacial dysmorphism and thumb abnormalities are classic in DBA. Short stature, urogenital abnormalities, web neck, skeletal, and cardiac defects can also present in patients with DBA. A classic description is that of Cathie: “tow-colored hair, snub nose, wide-set eyes, thick upper lips, and an intelligent expression.” Likewise, Aase and Smith described the triphalangeal thumb abnormality along with anemia seen in DBA.
Isolated anemia and reticulocytopenia in the presence of normal white cell count and platelet count are suggestive of PRCA. A review of the peripheral smear is the first step in the evaluation of PRCA. Further evaluation is pursued to determine the degree of anemia and the etiology of PRCA and rule out other diagnoses.
Untreated inherited PRCA results in severe anemia, which leads to congestive heart failure and death. Glucocorticoid use, blood transfusion, and allogeneic stem cell transplant (ASCT) is the mainstay of treatment in children.
Iron overload from chronic transfusions:
Any patient presenting with anemia and reticulocytopenia requires evaluation for PRCA. The low reticulocyte count helps differentiate PRCA from hemolytic anemia (which can also have isolated anemia, albeit with reticulocytosis).
The prognosis depends on the etiology of PRCA.
1. Inherited PRCA
2. Acquired PRCA:
Japanese consortium reported the long term follow-up results of patients with PRCA in their cohort of patients, which gave some insight into the long term prognosis of patients with acquired PRCA.
Children diagnosed with Diamond Blackfan anemia are predisposed to cancer. Although the risk of developing cancer is lower in patients with DBA compared to those diagnosed with Fanconi anemia, the risk is still higher than the general population belonging to the same age group. Reports exist of both solid and liquid malignancies with DBA in international registries. Acute myeloid leukemia is the most common hematologic malignancy, and osteogenic sarcoma is the most common solid organ malignancy diagnosed in children in DBA.
Chronic anemia can lead to growth retardation and poor psychomotor development in children. Severe anemia can also lead to a hyperdynamic circulation, which increases the work of heart and hence, the risk of cardiac failure, especially in those with pre-existing heart disease and the elderly. In pregnancy, it can prompt premature labor, abnormally low birth weight. In pregnant patients with severe anemia, there are reports of increased maternal and fetal mortality. Overall, anemia can cause low energy, constant fatigue, and in general, a poor quality of life.
Patients with PRCA who receive chronic transfusions eventually develop iron overload and related toxicities. Immunosuppressive therapy with cyclosporine or chronic steroid therapy can lead to myelosuppression and, eventually, opportunistic infections. The use of corticosteroids, whether short term high dose therapy or long term maintenance therapy, correlates with multiple adverse events.
Patients and physicians should understand the recurrent nature of the disease, the need for long term therapy, repeated blood transfusions, and iron chelation therapy. Any patient diagnosed with inherited PRCA should be evaluated for stem cell transplant at an early age, rather than performing it later, when the comorbidities from additional therapies have increased exponentially. A close follow-up of the affected patients should be kept to prevent complications from both the underlying disease as well as the treatment.
Pure red cell aplasia is a rare pathology that either presents as an inherited disease or that the patient acquires secondary to other conditions. Few pearls are listed here:
Pure red cell aplasia is a rare disorder. The inherited form is usually detected at birth, although many patients can also present in adolescent age or as young adults. It is imperative to maintain a broad outlook of the disease and investigate thoroughly starting with obtaining a good history, performing a thorough physical exam, and using appropriate clinical and genetic tests to determine the etiology.
Nurses, transfusion medicine, hematology, bone marrow transplant unit, and geneticist should work in close harmony with all clinicians as an interprofessional team to maximize the benefit to the patient. Patients managed with corticosteroids should be followed by the pharmacist to ensure that they are not developing complications. If there is any suspicion of osteoporosis, a bone scan is necessary. A pharmacist should examine the patient's medication record to look for drugs that may cause PCRA, as well as assist the clinician in any medication therapy undertaken in the management of the condition. These patients should also be followed by an infectious disease nurse, as they are prone to infections due to steroid therapy; the nurse can also monitor patient compliance and assess the effectiveness of the current treatment. The chronic blood transfusions can also result in iron overload, and hence the patient requires vigilant monitoring by the hematologist. These are examples of the interprofessional team approach to case management that optimizes positive patient outcomes. [Level V]
Due to the rarity of the pathology, no large randomized trials are possible, and the evidence of treatment comes from case series, retrospective studies of international registries, and few small prospective trials. [Level III to Level IV]
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