Patho blood & lymph

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• nvolvement of the marrow and peripheral blood Leukemia
• involvement of the lymph nodes (but also liver, spleen, BM)) Lymphomas
• Acute lymphoblastic leukemia/lymphoblastic lymphomas have Abrupt stormy onset (3months)
• Iron deficiency & thalassemia leads to Microcytic anemia which is associated with decreased MCV.
• Folate or B12 deficiency leads to macrocytic anemia and is associated with increased MCV.
• anemia of chronic disease, abnormal shapes (hereditary, spherocytosis, sickle cell), acute hemorrhage can lead to Normocytic anemia and is associated with an unchanged MCV.
• Shortness of breath (dyspnea), Organ failure, Shock is Acute Clinical symptoms
• Pallor, fatigue, lassitude- common to all forms of anemia. If hemolysis- hyperbilirubinemia, jaundice, gallstones If ineffective erythropoiesis: increased absorption of iron in gut- iron overload( secondary hemochromatosis), heart and endocrine failure If severe and congenital- growth retardation, bone deformation( due to reactive bone marrow hyperplasia), cachexia. are Chronic Clinical symptoms
• the most frequent cause of anemia is Iron deficiency and it can be caused by Chronic blood loss esp from GI (peptic ulcers, colon cancer) and female genitourinary tract( menorrhagia, metrorrhagia) , decreased retry intake or increased demand (e.g. pregnancy)
• The most common anemia in Hospitalized patients is Anemia of chronic disease and it's characterised by suppression of erythropoiesis (incr. hepcidin by pro-inflammatory cytokines which leads to downregulation of ferroportin in macrophages) and also decreased EPO synthesis.
• Accelerated RBC destruction, normal life span 120 days Normocytic or normochromatic. If blood loss is chronic→ depletion of body's iron storage, in 5-7 days compensatory rise in EPO levels. Compensatory inc in erythropoiesis→ inc in reticulocytes The retention of the products of degradation cells→ conversion of heme to bilirubin→ unconjugated hyperbilirubinemia, jaundice. These are the features of hemolytic anemia
• Occurs within the vascular compartment); hemoglobinuria, hemosiderinuria, hemoglobinemia. From mechanical force. Can lead to acute tubular necrosis. Intravascular hemolysis
• (Occurs within tissue macrophages)--> spleen and liver enlargement. More common. Extravascular hemolysis
• Hemolytic anemias are associated with erythroid hyperplasia in the marrow.
• hereditary spherocytosis is an intrinsic defects in RBC membrane that leads to formation of these types of RBCs. Mutations involve ankyrin band 3 and spectrin.A shared feature of the pathogenic mutations is that they weaken the vertical interaction between the membrane skeleton and the intrinsic membrane of protein. The critical role of the spleen is illustrated by the beneficial effect of splenectomy.
• The other general features of hemolytic anemias also are present, including cholelithiasis which occurs in 40-50% of patients with hereditary spherocytosis.
• The most common familial hemolytic anemia sickle cell anemia and is caused by mutations in the B-globulin gene.
• The presence of hemoglobins other than HbS influences the sickling of RBC. in vivo 40% is HbS, the rest is HbA – this is called sickle cell trait because HbA can retarde HbS. If you have HbC then kutt, because HbC has greater tendency to aggregate with HbS than any other causing HbSC.
• Thalassemia protects against malaria
• inherited disease in globin synthesis is called thalassemia and the mutations fall int 2 categories (1) Bo in which no B-globin chains are produced (2) B+ in which there is reduced B globin synthesis.
• Person inheriting one abnormal allele have B-thalassemia minor and are asymptomatic.
• People inheriting more than one allele have B thalassemia major
• Mutations leading to aberrant RNA splicing are the most common cause of B thalassemia.
• B thalassemia major manifests after birth when HbF synthesis diminishes.
• Heinz bodies & Bite cells G6PD deficiency
• G6PD is located on X-chromosome and has decreased half life –causing difficulty to restore reduced glutathione. Leading to RBC becoming more sensitive to oxidant stress
• intracellular inclusions of denatured hbg causes intravascular hemolysis Heinz bodies
• results from spleens attempt to pluck out Heinz bodies, such cells become trapped upon recirculation to the spleen and are destroyed by phagocytes (extravascular hemolysis). Bite cells
• Rare hemolytic disorder and the ONLY one that is ACQUIRED. It's caused by a mutation in PIGA gene leading to a mutation in the GPI protein found on the cell membrane. GPI usually binds to DAF proteins (which protects form immune complement). This anemia is known as Paroxysmal Nocturnal Hemoglobinuria
• Immunohemolytic anemias are uncommon and classified on the basis of The nature of the antibody & The presence of predisposing conditions
• Caused by obstruction of small vessels→ mechanical damage of RBC Many causes: disseminated intravascular congestion, malignant hypertension, SLE, hemolytic uremic syndrome, disseminated cancer. The anemia is Microangiopathic Hemolytic anemia
• Dysfunctional Bone marrow because of Suppression of multipotent myeloid stem cells, leading to BM failure and pancytopenia, Idiopathic/exposures to myelotoxic agents, radiation antineoplastic drugs, certain viral infections, inherited defects in telomerase, BM failure due to autoreactive T cells would lead to aplastic anemia and would show marked hypocellularity of marrow, Pancytopenia, dec reticulocytes and NO SPLENOMEGALY. But additionally Hemosiderosis & fatty change in the liver and intertrabecular spaces.
• Dysfunctional Bone marrow because of Replacement of marrow by infiltrative processes, Metastatic breast/lung/ prostate cancer, tuberculosis, lipid storage disorders, osteosclerosis would lead to Myelophthisic anemia and you would see that the WBC line is less affected, Early erythroid and granulocytic precursors and teardrop- shaped RBC peripherally .
• big, raspberry tongue Vit B12 ( CoBalamin) deficiency anemia
• Acute self limited disease caused by EBV infections is Infectious mononucleosis

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