Path: RBC, Bleeding Disorders Flashcards
Low ferritin/High TIBC/Low Hepcidin; Low serum Fe/Low % saturation; High RDW/High FEP; Low Hct/Low Hb
Iron-Deficiency Anemia (IDA)
Associated Illness with IDA
Plummer-Vinson Syndrom (Anemia, Esophageal Webs, Dysphagia)
Increased IL-6 APP –> Increased Hepcidin –> decreased Ferroportin and EPO
Anemia of Chronic Disease
High ferritin/low TIBC; low serum Fe/Low % saturation; High FEP; low EPO
Anemia of Chronic Dz
Deletion in Chr. 16
Alpha Thalassemia
Mutation in Chr. 11
Beta Thalassemia
Hb Barts
Gamma tetramers, 4 gene deletion in Alpha Thalassemia
HbH
Beta tetramers, 3 gene deletion in Alpha Thalassemia
HbF > HbA2
B-Thal Major
HbA2 > HbF
B-Thal Minor
Low RBC, hypersegmented Neutrophils, MCV >100
Megaloblastic Anemia
Incr. Homocysteine, Normal Methymalonic Acid, MCV >100
Folate Deficiency
Incr. Homocysteine, Incr. Methylmalonic Acid, MCV >100
B12 Deficiency
AI Atrophic Gastritis –> AI T-cells
Pernicious Anemia –> B12 deficiency
Ankyrin, Spectrin, Band3, Howell-Jolly Bodies
Hereditary Spherocytosis (Extravascular)
HbS
Valine substitution –> Sickle-Cell Anemia
HbSC
Valine & Lysine substitutions –> mild sickling
PIGA –> GPI proteins (MIRL, DAF, C8 binding protein)
Paroxysmal Nocturnal Hemoglobinuria (PNH) = intravascular hemolysis
Heinz Bodies, Bite Cells; X-linked recessive; intermittent hemolysis
G6PD Deficiency
Warm Agglutinins
IgG
Cold Agglutinins
IgM
D-dimers, Schistocytes
Microangiopathic Hemolytic Anemia
Fanconi Anemia
Aplastic Anemia
Parvovirus B19
Pure Red Cell Aplasia (decreases RBCs)
Space occupying lesion –> suppressed BM –> Tear-drop RBC and Leukoerythroblastosis (immature granulocytes and RBC)
Myelophthistic Anemia
Uremia + Extracorpuscular defects + Decreased EPO synthesis –> underproduce RBCs
Chronic Renal Failure
Gaisbock Syndrome
Stress Polycythemia = reduced plasma volume with HTN, obese, anxious patient
Stabilized HIF1-alpha –> increased EPO
Secondary Polycythemia
PTT, Heparin pathway (less important)
Intrinsic
PT, Tissue damage, Coumadin pathway (most important)
Extrinsic
Purpuric rash, colic, polyarthralgia, acute glomerulonephritis
Henoch-Schonlein Purpura
TGF-B; tortuous veins, epistaxis and other very serious bleeding complications
Weber-Osler-Rendu Syndrome
Peripheral Megathrombocytes, Increased BM megakaryocytes, large peripheral platelets, no splenomegaly or LAD; petechial hemorrhages
Chronic Immune Thrombocytopenic Purpura (hemorrhage)
Follicular B-cell hyperplasia –> Auto-Abs to GPIIb/IIIa
HIV-associated Thrombocytopenia
ADAMTS13 –> excessive platelet activation
Thrombotic Thrombocytopenic Purpura (thromboses)
E.Coli activated thrombotic microangiopathy
Hemolytic Uremic Syndrome (HUS)
GPIb-Ix (Aut. Rec.) –> Platelet Adhesion defect
Bernard-Soulier Syndrome
GPIIb/IIIA (Aut. Rec.) –> Platelet Aggregation defect
Glanzmann Thrombasthenia
Prolonged PTT
vWF, Hemophilia A/B
ADAMTS13 + normal platelets, prolonged PTT, hemarthoris
vWF (Type 3) = Aut. Recessive
Factor VIII deficiency; hemarthorses; prolonged PTT
Hemophilia A (X-linked Recessive)
Factor IX deficiency; hemarthroses; prolonged PTT
Hemophilia B (X-linked recessive)
Non-immunologic Thrombocytopenia, MAJOR bleeding, triggered by Tissue Factor; hemorrhage and thromboses
DIC
IgG reaction to IgA in donor blood
IgA deficiency
Acute Transfusion hemolysis
IgG
Delayed Transfusion hemolysis
IgM
Acute Respiratory Depression, Hypotension, hypoxemia, increased activated neutrophils AFTER transfusion
TRALI