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8 - Clinical presentation, natural course, and prognostic factors

from Part II - Epidemiology and clinical features of acquired aplastic anemia

Published online by Cambridge University Press:  18 August 2009

Pedro Marín-Fernandez
Affiliation:
Farreras Valenti Hospital Clinic, Barcelona
Hubert Schrezenmeier
Affiliation:
Freie Universität Berlin
Andrea Bacigalupo
Affiliation:
Ospedale San Martino, Genoa
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Summary

Clinical manifestations

Medullary aplasia does not have specific signs and symptoms, and its clinical manifestations are similar to those observed in other oncohematological diseases such as acute leukemia, some types of lymphomas, myelosclerosis, myelodysplasia, and following chemotherapy or radiotherapy treatment. They are caused by hemopoietic failure and the intensity of expression is directly related to the degree of pancytopenia.

The patient usually consults with an anemic syndrome together with skin or mucosal hemorrhages (Marín, 1981:114; Rozman et al., 1981:321; Scott et al., 1959:119; Williams et al., 1973:195). Patients present less frequently with an isolated infection or anemic syndrome as the initial manifestation of the disease (Marín, 1981:116; Rozman et al., 1981:321). Disease perception may vary considerably. While some patients are diagnosed during a routine health check-up and are practically asymptomatic, others present an acute clinical situation. This explains why the time that elapses between the initial presentation of symptoms and medical attention varies considerably, from days to years, reflecting the behavior and severity of the disease (Lynch, 1975:517; Marín, 1981:115; Rozman et al., 1981:321; Williams et al., 1973:195). The anemic syndrome, together with hemorrhages, is the most frequently described clinical presentation, which in half of the cases is accompanied by hemodynamic symptoms that may disclose prior cardiovascular abnormalities (Marín, 1981:114). Hemorrhagic symptoms are usually slight and easily controlled, and manifest as ecchymoses, petechiae, epistaxis or visual field defects. However, in 20% of cases they can be severe and cause death.

Type
Chapter
Information
Aplastic Anemia
Pathophysiology and Treatment
, pp. 117 - 134
Publisher: Cambridge University Press
Print publication year: 1999

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