Anemia 3 - Lecture notes 3 PDF

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Summary

Bone marrow failure Aplastic Anemia Pure Red Cell Aplasia Congenital Dyserythropoietic Anemia Myelophthisic Anemia Anemia of Chronic Kidney Disease Bone marrow failure: reduction of cessation of blood cell production affecting one or more cell lines. o Pancytopenia is always seen Destruction of hema...

Description

HEMATOLOGY Aplastic anemia

Bone marrow fai failure lure -

Aplastic Anemia Pure Red Cell Aplasia Congenital Dyserythropoietic Anemia Myelophthisic Anemia Anemia of Chronic Kidney Disease

History of Aplastic Anemia -

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Bone marrow failure: reduction of cessation of blood cell production affecting one or more cell lines. o Pancytopenia is always seen

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Destruction of hematopoietic stem cells

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drugs, chemicals, radiation, viruses or autoimmune mechanisms.

2. Premature senescence and apoptosis of HSCs - mutations 3. Ineffective hematopoiesis: stem cell mutations and Vit B12 or Folate deficiency 4. Decreased production of hematopoietic GF 5. Disruption of BM microenvironment that supports hematopoiesis. 6. Loss of normal hematopoietic tissue due to infiltration of abnormal cells

Characterized by: thrombocytopenia: significant bleeding, decreased RBC count and Hgb: fatigue, neutropenia: risk of bacterial and fungal infections

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Paul Ehrlich (1854-1915) described the first case of aplastic anemia in a pregnant woman who died of marrow failure in1888. A fatal BM syndrome o Pancytopenia, reticulocytopenia, BM hypocellularity and depletion of HSCs The term “aplastic anemia” was given to the disease by Vaquez and Aubertin in 1904.

Acquired aplastic anemia -

More common 70% idiopathic 10-15% secondary Macrocytic or normocytic anemia with reticulocytopenia

etiolo etiology gy Etiology Acquired: (idiopathic, secondary to) Drugs Agents with predictable, doserelated effects (e.g., chemotherapeutic drugs) - Agents with unpredictable, nondose-related effects (e.g., chloramphenicol) o Cytotoxic drugs o Antibiotics -

HEMATOLOGY Chloramphenicol Anti-inflammatory Anti-convulsant Sulphonamides 2-3 months usually between exposure and the development of aplastic anemia. Supress BM Radiations Chemicals e.g., Benzene and pesticides Viruses: o Hepatitis A, Non-A and NonB o Herpes simplex, E-B virus o Parvovirus: Transient o 2-3 months between exposure to the virus and the development of AA. Immune: SLE, RA (rheumatoid arthritis), Pregnancy Idiopathic: 70% o o o o o

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Severe depletion of HSC and Progenitor cells may be due to: 1. Direct damage to stem cells: drugs, chemicals etc. 2. Immune mechanism damage to stem cells

clinical findings Clinical findings -

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CD 34 cells 10x lower than that with healthy people.

Anemia: pallor, fatigue and weakness Severe anemia: cardiac complications, cardiac failure and death Petechiae, bruising, epistaxis, bleeding gums, menorrhagia and bleeding disorders. Fever and bacterial infectionsneutropenia

laboratory findings findings Laboratory -

pathophysiolog pathophysiologyy Pathophysiology

Production of GF is normal-support growth of CD34 cells. EPO, TPO, G-CSF, GM-CSF all increase-compensate pancytopenia

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Pancytopenia Hgb...