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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 1) ppsx

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Chapter 102. Aplastic Anemia, Myelodysplasia, and
Related Bone Marrow Failure Syndromes
(Part 1)

Harrison's Internal Medicine > Chapter 102. Aplastic Anemia,
Myelodysplasia, and Related Bone Marrow Failure Syndromes
Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure
Syndromes: Introduction
The hypoproliferative anemias are normochromic, normocytic or
macrocytic and are characterized by a low reticulocyte count. Deficient production
of RBCs occurs with marrow damage and dysfunction, which may be secondary to
infection, inflammation, and cancer. Hypoproliferative anemia is also a prominent
feature of hematologic diseases that are described as bone marrow failure states;
these include aplastic anemia, myelodysplasia (MDS), pure red cell aplasia
(PRCA), and myelophthisis. Anemia in these disorders is often not a solitary or
even the major hematologic finding. More frequent in bone marrow failure is
pancytopenia: anemia, leukopenia, and thrombocytopenia.
Low blood counts in the marrow failure diseases result from deficient
hematopoiesis, as distinguished from blood count depression due to peripheral
destruction of red cells (hemolytic anemias), platelets (idiopathic
thrombocytopenic purpura or due to splenomegaly), and granulocytes (as in the
immune leukopenias).
Hematopoietic failure syndromes are classified by dominant morphologic
features of the bone marrow (Table 102-1). While practical distinction among
these syndromes usually is clear, they can occur secondary to other diseases, and
some processes are so closely related that the diagnosis may be complex.
Patients may seem to suffer from two or three related diseases
simultaneously, or one diagnosis may appear to evolve into another. Many of these
syndromes share an immune-mediated mechanism of marrow destruction and
some element of genomic instability resulting in a higher rate of malignant
transformation.



Table 102-1 Differential Diagnosis of Pancytopenia


Pancytopenia with Hypocellular Bone Marrow

Acquired aplastic anemia
Constitutional aplastic anemia (Fanconi's anemia, dyskeratosis congenita)
Some myelodysplasia
Rare aleukemic leukemia (AML)
Some acute lymphoid leukemia
Some lymphomas of bone marrow
Pancytopenia with Cellular Bone Marrow

Primary bone marrow diseases
Myelodysplasia

Paroxysmal nocturnal
Secondary to systemic
diseases

Systemic lupus
hemoglobinuria
Myelofibrosis
Some aleukemic leukemia
Myelophthisis
Bone marrow lymphoma
Hairy cell leukemia
erythematosus
Hypersplenism

B
12
, folate deficiency
Overwhelming infection
Alcohol
Brucellosis
Sarcoidosis
Tuberculosis
Leishmaniasis
Hypocellular Bone Marrow ± Cytopenia

Q fever
Legionnaires' disease
Anorexia nervosa, starvation
Mycobacteria


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