Presentation on theme: "APLASTIC AND HYPOPLASTIC ANEMIAS"— Presentation transcript:
1APLASTIC AND HYPOPLASTIC ANEMIAS What happens when the bone marrow shuts down?
2APLASTIC ANEMIAAplastic anemia is a severe, life threatening syndrome in which production of erythrocytes, WBCs, and platlets has failed.Aplastic anemia may occur in all age groups and both genders.The disease is characterized by peripheral pancytopenia and accompanied by a hypocellular bone marrow.
4APLASTIC ANEMIA Pathophysiology: The primary defect is a reduction in or depletion of hematopoietic precursor stem cells with decreased production of all cell lines. This is what leads to the peripheral pancytopenia.This may be due to quantitative or qualitative damage to the pluripotential stem cell.In rare instances it is the result of abnormal hormonal stimulation of stem cell proliferationor the result of a defective bone marrow microenvironmentor from cellular or humoral immunosuppression of hematopoiesis.
6APLASTIC ANEMIA Etiology Acquired Most cases of aplastic anemia are idiopathic and there is no history of exposure to substances known to be causative agents of the diseaseExposure to ionizing radiation – hematopoietic cells are especially susceptible to ionizing radiation. Whole body radiation of rads can completely wipe out the bone marrow. With sublethal doses, the bone marrow eventually recovers.Chemical agents – include chemical agents with a benzene ring, chemotherapeutic agents, and certain insecticides.Idiosyncratic reactions to some commonly used drugs such as chloramphenicol or quinacrine.
7APLASTIC ANEMIAInfections – viral and bacterial infections such as infectious mononucleosis, infectious hepatitis, cytomegalovirus infections, and miliary tuberculosis occasionally lead to aplastic anemiaPregnancy (rare)Paroxysmal nocturnal hemoglobinuria – this is a stem cell disease in which the membranes of RBCs, WBCs and platlets have an abnormality making them susceptible to complement mediated lysis.Other diseases – preleukemia and carcinoma
8APLASTIC ANEMIA Congenital disorders Fanconi’s anemia – the disorder usually becomes symptomatic ~ 5 years of age and is associated with progressive bone marrow hypoplasia. Congenital defects such as skin hyperpigmentation and small stature are also seen in affected individuals.Familial aplastic anemia – a subset of Fanconi’s anemia in which the congenital defects are absent.Clinical manifestationsFatigueHeart palpitationsPallorInfectionsPetechiaeMucosal bleeding
9APLASTIC ANEMIA Lab findings Severe pancytopenia with relative lymphocytosis (lymphocytes live a long time)Normochromic, normocytic RBCs (may be slightly macrocytic)Mild to moderate anisocytosis and poikilocytosisDecreased reticulocyte countHypocellular bone marrow with > 70% yellow marrowTreatment – in untreated cases the prognosis is poorRemove causative agent, if knownMultiple transfusionsBone marrow transplant
10RELATED DISORDERSDisorders in which there is peripheral pancytopenia, but the bone marrow is normocellular, hypercellular, or infiltrated with abnormal cellular elementsMyelopthesic anemia – replacement of bone marrow by fibrotic, granulomatous, or neoplastic cells
11RELATED DISORDERSMyelodysplastic syndromes – are primary, neoplastic stem cell disorders that tend to terminate in acute leukemia. The bone marrow is usually normocellular, or hypercellular with evidence of qualitative abnormalities in one or more cell lines resulting in ineffective erythropoiesis and/or granulopoiesis and/or megakaryopoiesis. The peripheral smear shows dysplastic (abnormality in development) cells including nucleated RBCs, oval macrocytes, pseudo-Pelger-Huet PMNs (hyposegmented neutrophils) with hyperchromatin clumping, hypogranulated neutrophils, and giant bizarre platlets.Hypersplenism – why can this lead to pancytopenia?
12PURE RED CELL APLASIAPure red cell aplasia is characterized by a selective decrease in erythroid precursor cells in the bone marrow. WBCs and platlets are unaffected.AcquiredTransitory with viral or bacterial infectionsPatients with hemolytic anemias may suddenly halt erythropoiesisPatients with thymoma – T-cell mediated responses against bone marrow erythroblasts or erythropoietin are sometimes produced.
13PURE RED CELL APLASIA Congenital Diamond-Blackfan syndrome – occurs in young children and is progressive. It is probably due to an intrinsic or regulatory defect in the committed erythroid stem cell.
14OTHER HYPOPROLIFERATIVE ANEMIAS Renal disease – due to decreased erythropoietinEndocrine deficiencies – may lead to decreased erythropoietin production. For example: hypothyroidism leads to decreased demand for oxygen from tissues; decreased androgens in males; decreased pituitary function