Step 1 - HemeOnc 1

denniskwinn's version from 2015-04-25 16:02


Question Answer
Fetal erythropoiesis happens in the (3-8 wks)Yolk Sac [what happens here during embryogenesis and during what weeks?]
Fetal erythropoiesis happens in the (6-30 wks)Liver [what happens here during embryogenesis and during what weeks?]
Fetal erythropoiesis happens in the (9-28 wks)Spleen [what happens here during embryogenesis and during what weeks?]
Fetal erythropoiesis happens in the (28+ wks)Bone Marrow [what happens here during embryogenesis and during what weeks?]
Fetal hemoglobinα2ɣ2
Adult Hemoglobin α2β2


Question Answer
Final cell types for Myeloid stem cell (6)Erythrocyte, Nphil, Eosinophil, Basophil, Platelets, Monocyte
Final cell types for Lymphoid stem cell (3)Plasma cell, T cell, NK cell
RBC differentation path (5)Pluripotent hematopoetic SC→ Myeloid SC→ Proerythroblast→ Reticulocyte→ Erythrocyte
Nphil/Eosino/Basophil differentiation path (8)Pluripotent hematopoetic SC→ Myeloid SC→ Myeloblast → Promyelocyte (of N/E/B lineage) → myelocyte→ metamyelocyte→stab(band) cell→ lineage for x type (N, E or B)
Platelet differentiation path (5)Pluripotent hematopoetic SC→ Myeloid SC→megakaryoblast megakaryocyteplatelets
Monocyte differentiation path (4) Pluripotent hematopoetic SC→ Myeloid SC→ monoblast → monocyte
Plasma/T cell differentiation path (5) Pluripotent hematopoetic SC→ Lymphoid SC → Lymphoblast → T or B cell→ Active T or Plasma cell
NK cell differentiation path (4) Pluripotent hematopoetic SC→ lymphoid SC → Pro NK →NK cell
Erythrocytosis = polycythemia ↑ number of red cells.
Anisocytosisvarying sizes. [definition of?]
Poikilocytosisvarying shapes. [definition of]
Reticulocyteimmature RBC
RBC survival time120 days [life span of?]
RBC metabolismGlucose anaerobically degraded - 90% to lactate, 10 % HMP shunt
RBC membraneContains chloride-bicarbonate antiport which allows CO2 transports from periphery to lungs (“physiologic chloride shift”)
Thrombocytopenialow platelets [definition] - results in petechiae
Platelet life span8-10 days [life span of?]


Question Answer
Granulocyte types (3)Basophil, Eosinophil, Neutrophil
Normal leukocyte count 4-10k/microliter
Basophils (4)1.mediates allergic rxn
2. bilobate nucleus
3. Contain heparin, histamine and leukotrienes
4. Found in blood
Mast cells (4)1. Mediates allergic rxn
2. Degranulates releasing: histamine, heparin, and eosinophilic chemotactic factors
3. Binds IgE fcto membrane
4. Type I hypersensitivity
Cromolyn sodiumPevents mast cell degranulation (used as a prophylatic for asthma)
Eosinophil (4)1. Packed with large eosinophilic granules
2. Defends against helminthic and protozoaninfections.
3. Highly phagocytic for antigen-antibody complexes.
4. Produces histaminase and arylsulfatase (help limit rxn following mast cell degranulation)
Causes of eosinophilia (5)NAACP - Neoplastic, Asthma, Allergic processes, Collagen vascular diseases, Parasites (invasive)
Neutrophil (4)1. Acute inflammatory response cell
2. Phagocytic
3. Multi-lobed nucleus
4. Contain hydrolytic enzymes, lysozyme, myeloperoxidase and lactoferrin
Hypersegmented polys are seen invitamin B12/folate deficiency [what you see on periph smear]
Monocoytes (2)1. Differentiates into macrophages in tissues
2. Large kidney shaped nucleus
Macrophage (4)1. Phagocytosis of bact, debris
2. Long life in tissues
3. Activated by ɣ-interferon
4. Can function as APC via MHC II
Dendritic cells (4)1. MHC
2. Express MHC II and FcR
3. Main inducers of primary antibody response.
4. Called Langerhans cells on skin
Lymphocyte (2)1. Round, densly staining nucleus.
2. B and T cells
B lymphocyte (4)1. Matures in marrow
2. Migrates to peripheral lymph tissue (follicles of LN)
3. When antigen is encountered B cells differentiate into plasma cells and produce antibodies
4. Can function as an APC via MHC II
Plasma cell (2)1. Off center, clock-face chromatin distribution
2. B cells differentiate into plasma cells which produce large amounts of antibody specific to a particular antigen
T cells (3)1. Mediate cellular immune response
2. Mature in thymus into cytotoxic T, helper T and suppressor T
MHC II =CD4 [what MHC]


Question Answer
Outcomes of incompatible blood transfusionsCan cause immunologic response: hemolysis, renal failure, shock and death
- anti-AB antibodies are IgM so they can’t cross the placenta
- anti-Rh antibodies are IgG and can [outcome of]
Rh factor- Indicates the presence of antigen
-Rh- indicates absence
- Rh- mom can be exposed to Rh+ fetus and can make anti-Rh IgG that can cross the placenta during subsequent pregnancy causing hemolytic disease of the newborn.
Hemophilia A deficiency of Factor VIII
Hemophilia B deficiency of Factor IX
Vitamin K deficiency(6)↓ synthesis of factors II, VII, IX, X, protein C, protein S [deficiency of?]
Learn coagulation cascade make coagulation cascade image based thinger and thrombogenesis (formation of insoluble fibrin mesh)
Platelet plug adhesionvWF mediates linking of platelet Gp1b receptor to subendothelial collagen [allows for]
Platelet plug aggregation Represents a balance between pro-aggregation and anti-aggregation factors:
-TXA2 released by platelets increasing aggregation.
-PGI2 and NO released by endothelial cells decrease aggregation
Platelet plug swelling-Binding of ADP on platelet receptors=induces expression of GpIIb/IIIa on platelet membrane . GpIIb/IIIa binds fibrinogen and links platelets=allowing platelet cohesion.
-Ca2+ also strengthens platelet plug
Aspirin inhibitsCOX1- inhibiting TxA2 synthesis
Ticlopidine and clopidogrel inhibitisADP-induced expression of GpIIb/IIIa [inhibited by]
Abciximab inhibitsGpIIb/IIIa directly [inhibited by]
Pro-coagulationVitamin K gets activated by epoxide reductase and is a cofactor for factor II, VIII, IX, X, C, S
Warfarin inhibitsEpoxide reductase - preventing activation of vitamin K and the factors that it helps [a true fact; just not in FA 2011 Hem-Onc Chapter]
Neonates lack enteric bacteria - making problems with coagulation - so they can’t produce vitamin K
Antithrombin inactivates (5)[basically: Factors 2, 7, 9-12 inhibited by]
Antithrombin is activated byheparin [activates]
tPAturns plasminogen to plasmin - which cleaves fibrin mesh


Question Answer
Acanthocyte(aka spur cell) RBC
- Liver disease
- Abetalipoproteinemia [what you see on periph smear]
Basophilic stippling seen in (4)1.Thalassemias
2. Anemia of chronic disease
3. Iron deficiency
4. Lead poisoning
-(Baste the ox taill)
Bite cell seen inG6PD deficiency [what you see on periph smear]
Elliptocyte cell seen inHereditary elliptocytosis [what you see on periph smear]
Macro-ovalocyte seen in (2)-Megaloblastic anemia (also hypersegmented PMNs) -Marrow failure
Ringed sideroblasts seen inSideroblastic anemia [what you see on periph smear]
Schistocyte, helmet cells seen in (3)-DIC
-Traumatic hemolysis [what you see on periph smear]
Spherocytes seen in (2)Hereditary spherocytosis
-Autoimmune hemolysis [what you see on periph smear]
Teardrop cells seen inBM infiltration (myelofibrosis) [what you see on periph smear]
Target cells seen in (4)HbC disease, Asplenia, Liver disease, Thalassemia
(Halt said the hunter to his targer)
Heinz bodies assoc with (2)-Alpha-thalassemia
-G6PD deficiency [what you see on periph smear]
Heinz bodies processOxidation of iron from ferrous to ferric form leads to denatured hemoglobin precipitation and damage to RBC membrane=Leads to formation of bite cells
Howell-Jolly bodies seen inPatients w/functional hyposplenia or asplenia [what you see on periph smear]
Howell-Jolly bodies processBasophilic nuclear remnants found in RBCs


Question Answer
Iron deficiency anemia(microcytic) 1. ↓ Iron due to: chronic bleeding, malnutrition/absorption disorders or ↑demand (preggers) →↓heme synth
Plummer-Vinson syndromeTriad
1)Iron deficiency anemia
2)Esophageal web
3)Atrophic glossitis
Alpha thallassemia(microcytic)
1. Defect: alpha globin gene mutation→ alpha globin synth.
2. Prevalent in Asia and Africa
3. Deletion of 4 genes incompatible with life→Ɣ4 globin = hydrops fetalis
4. deletion of 3 = HbH disease
5. 1-2 deleted asx - mild anemia
Beta thalassemia (3)(microcytic)
1. Defect: point mutations or promotor regions mutations
2. Prevalent in Mediterranean populations.
3. Heterozygote = minor, homozygote = major
Beta thalassemia minor(3)(microcytic)
1. Beta chain underproduced
2. Usually asx
3.Diagnosis by increases HbA2>3.5%
4. Also ↑ HbF (alpha2gamma2)
Beta thalassemia major(3)(microcytic)
1. Beta chain absent→severe anemia requiring blood transfusion (secondary hemochromatosis)
2. Marrow expansion (“crew cut” on skull x-ray)→skeletal deformaties - chipmunk facies
3. ↑HbF(alpha2gamma2)
HbS/B-thalassemia heterozygote mild to moderate sickle cell disease - depending on amount of B-globin production


Question Answer
Lead poisoning(2)(microcytic)
1. inhibits ferrochelatase and ALA dehydratase↓heme synthesis.
2. Also inhibits RNA degradationbasophilic stippling
Sideroblastic anemia (6)(microcytic)
1. Defect in heme synthesis
2. X-linked defect in delta-aminolevulinic acid synthase gene
3. Tx w/pyridoxine (B6) therapy
4. Reversible etiologies: from alcohol and lead.
5. ↑ Iron, normal TIBC, ↑Ferritin
6. Smear shows ringed sideroblasts (with iron laden mitochondria)
Megaloblastic anemia ↓ folate findings (4)(macrocytic)
1. Hypersegmented neutrophils
2. Glossitis,
4. ↑homocysteine but normal methylmalonic acid
Megaloblastic anemia ↓ folate etiologies (4)1. Malnutrition(alcohol)
2. Malabsorption
3. Impaired metab (methotrexates, trimethoprim)
4.↑ requirement (hemolytic anemia, pregnancy)
Megaloblastic anemia ↓B12 findings (4)(macrocytic)
1. Hypersegmented neutrophils
2. Glossitis
3. ↑homocysteine
4. ↑methylmalonic acid (neuro degenerative sx)
Megaloblastic ↓ B12 etiologies1. Malnutrition (alcohol)
2. Malabsorption (crohn’s,Pernicious anemia)
3. Diphyllobothrium latum (fish tapeworm)
Nonmegaloblastic macrocytic anemias (5)1. Liver disease
2. Alcoholism: macrocytosis & BM suppression w/out deficiency of B12/folate
3. Reticulocytosis: reticulocytes are bigger than mature RBCs→↑MCV
4. Metabolic disorder (orotic aciduria): congen deficiencies of purine or pyrimidine synth
5. Drugs: 5-FU, AZT, Hydroxyurea
Nonhemolytic normocytic, normochromic anemia (3)1. Anemia of chronic disease (ACD)
2. Aplastic anemia
3. Kidney disease
Intrinsic hemolytic normocytic, normochromic anemia (6)1.Hereditary spherocytosis
2. G6PD deficiency
3. Pyruvate kinase deficiency
4. Sickle,
5. HbC defect
6. PNH
Extrinsic hemolytic normocytic, normochromic anemia (3)1. Autoimmune
2. Microangiopathic (DIC, TTP-HUS)
3. Infection
DICDisseminated intravascular coagulation [abbreviation]
TTP-HUSThrombotic Thrombocytopenic Purpura and Hemolytic-Uremic Syndrome [abbreviation]