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Chapter 30  Aplastic Anemia  395












                          A










                          B                         C                         D
                            Fig. 30.1  BONE MARROW MORPHOLOGY IN SEVERE APLASTIC ANEMIA. Bone marrow biopsy
                            specimen, of sufficient length (A) shows severe hypocellularity. The corresponding aspirate (B, D) shows empty
                            marrow spicules and residual stoma including lymphoid cells, plasma cells, histiocytes and mast cells.



             TABLE   Differential Diagnosis of Pancytopenia        TABLE   A Classification of Aplastic Anemia
              30.1                                                  30.2
             Pancytopenia With Hypocellular Bone Marrow            Acquired Aplastic Anemia
             Acquired aplastic anemia                              Secondary aplastic anemia
             Inherited aplastic anemia (Fanconi anemia and others)  Irradiation
             Some myelodysplasia syndromes                         Drugs and chemicals
             Rare aleukemic leukemia (acute myelogenous leukemia)  Regular effects
             Some acute lymphoblastic leukemias                    Cytotoxic agents
             Some lymphomas of bone marrow                         Benzene
             Pancytopenia With Cellular Bone Marrow                Idiosyncratic reactions
             Primary bone marrow diseases                          Chloramphenicol
             Myelodysplasia syndromes                              Nonsteroidal antiinflammatory drugs
             Paroxysmal nocturnal hemoglobinuria                   Antiepileptics
             Myelofibrosis                                         Gold
             Some aleukemic leukemias                              Other drugs and chemicals
             Myelophthisis                                         Viruses
             Bone marrow lymphoma                                  Epstein-Barr virus (infectious mononucleosis)
             Hairy cell leukemia                                   Hepatitis virus (non-A, non-B, non-C, non-G hepatitis)
             Secondary to systemic diseases                        Parvovirus (transient aplastic crisis, some pure red cell aplasia)
             Systemic lupus erythematosus, Sjögren syndrome        Human immunodeficiency virus (acquired immunodeficiency syndrome)
             Hypersplenism                                         Immune diseases
             Vitamin B 12 , folate deficiency (familial defect)    Eosinophilic fasciitis
             Overwhelming infection                                Hyperimmunoglobulinemia
             Alcohol                                               Thymoma and thymic carcinoma
             Brucellosis                                           Graft-versus-host disease in immunodeficiency
             Ehrlichiosis                                          Paroxysmal nocturnal hemoglobinuria
             Sarcoidosis                                           Pregnancy
             Tuberculosis and atypical mycobacteria                Idiopathic aplastic anemia
             Hypocellular Bone Marrow ± Cytopenia                  Inherited Aplastic Anemia
             Q fever                                               Fanconi anemia
             Legionnaires disease                                  Dyskeratosis congenita
             Mycobacteria                                          Shwachman-Diamond syndrome
             Tuberculosis a                                        Reticular dysgenesis
             Anorexia nervosa, starvation                          Amegakaryocytic thrombocytopenia
             Hypothyroidism                                        Familial aplastic anemias
                                                                   Preleukemia (e.g., monosomy 7)
             a Pancytopenia in tuberculosis only rarely is associated with a hypocellular bone
             marrow at biopsy or autopsy. Marrow failure in the setting of tuberculosis is   Nonhematologic syndromes (e.g., Down, Dubowitz, Seckel)
             almost always fatal; exceptional patients probably had underlying
             myelodysplasia or acute leukemia.
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