RBC and Bleeding Flashcards
Main hematopoietic organ at 3rd week
yolk sac
Main hematopoietic organ at 3rd month
liver
Main hematopoietic organ at 4th month
BM
Hematopoietic organs at birth:
Mainly BM.
Liver is minimal
Normal Hb for male? female?
13-16 g/dl
12-14
Normal Hct for male? female?
39-48%
33-42%
What are the target molecules in chronic ITP?
glycoprotein IIb-IIa
or
glycoprotein Ib-IX
Normal MCV:
80 <, Normal < 94
Normal MCHC
Normal >= 32
This is a thrombo-hemorrhagic disorder resulting from a complication of a variety of diseases. It is characterized by excessive activation of coagulation, leading to the formation of thrombi in the microvasculature of the body.
Disseminated intravascular coagulopathy
Autoantibody blocks B12-IF complex to ileal receptor describes what anemia?
Type II anti-IF pernicious anemia
Main symptoms of hereditary spherocytosis
anemia; splenomegaly & jaundice
This viral infection can cause aplastic crisis in SC anemia and spherocytosis.
Parvovirus B19
IgG-coated RBC binds to monocytes and splenic macrophages. Non-complement. What type of IHA?
Warm agglutinin IHA (Warm is “G”reat weather)
Two major variants in G6PD deficiency we studied:
G6PD A- (only mature lack) G6PD Mediterranean (all RBC lack)
Hemolysis in G6PD def is intra- or extravascular?
both
Hemolytic anemia resulting from trauma to RBCs from narrowing or obstruction of microvasculature
microangiopathic hemolytic anemia
RBCs lysed in the circulation releasing hemoglobin into the plasma describes what kind of hemolysis?
intravascular
Massive destruction of RBC passing through spleen causing SUDDEN splenomegaly leading to hypovolemic shock
Sequestration crisis
Complement-mediated hemolytic anemia characterized by increased urine hemosiderin and thrombosis.
Paroxysmal nocturnal hemoglobinuria.
Cooley’s anemia is associated with what (specific) anemia?
Beta-thalassemia major
Presence of abnormal Hb due to substitution of valine for glutamine at 6th position of Beta chain which leads to low O2 or dehydration precipitates describes which disease’s pathogenesis:
sickle cell anemia
Crew cut appearance on xray
sickle cell anemia
Beta thalassemia major
Coomb’s test is a major diagnostic criterion for which group of anemias?
immunohemolytic anemias (tests antiglobulin)
Impaired DNA synthesis –> maturation of nucleus delayed relative to cytoplasm –> ineffective erythropoiesis resulting in pancytopenia. This describes the pathogenesis of:
Megaloblastic anemia
What causes the crew cut appearance of skull x-ray?
marrow expansion from increased erythropoeisis
Back pain, hemoglobinuria a few days later.
PBS: bite cells and Heinz bodies
G6PD deficiency
Multiple simultaneous painful thrombosis in different organs (dactylitis and acute chest syndrome seen) is known as what? This is a complication of which disease?
vaso-occlusive crisis
sickle cell anemia
In G6PD deficiency, what can cause oxidant stress leading to hemolytic anemia:
sulfa drugs
infections
fava beans
Which membrane proteins are deficient because of a defective PIGA gene?
CD59 (lysis inhibitor)
CD55 (DAF)
and C8
Hemoglobin electrophoresis revealed No HbA, but marked elevation in HbF/HbA2. What anemia does this person have?
Beta-thalassemia major
Hemoglobin electrophoresis revealed 4 beta chains. What anemia does this person have?
alpha thalassemia (HbH)
IgM-binds to RBC. Complement activated.
Cold agglutinin IHA (RBC binding at 0-4 degrees C)
Hemoglobin electrophoresis revealed decreased HbA and slight elevation in HbA2 (>3.5%)? What anemia is this?
beta thalassemia minor
This anemia results from an abnormal PIGA gene.
Paroxysmal nocturnal hemoglobinuria
Which microcytic, hypochromic anemia is prevalent in Asian and African populations:
alpha-thalassemia
Sudden failure of bone marrow to compensate with RBC production despite anemia
aplastic crisis
Chronic anemia seen in SLE, CLL, or with certain drugs:
Warm agglutinin IHA
Defective proteins in spherocytosis:
ankyrin, spectrin, band 3, protein 4.2
Hemoglobin electrophoresis revealed 4 gamma chains. What anemia?
alpha thalassemia (Barts)
Most common causes: Mycoplasma pneumoniae infections or infectious Mononucleosis
Cold agglutinin IHA
COLD icecream = MMM ….. IgM, mycoplasma, mono
IgG binds to RBC in cold temp. Hemolysis occurs when blood returns to warmer areas.
Paroxysmal cold hemoglobinuria ( Donath-Landsteiner syndrome)
or Cold hemolysin IHA
Common causes of Donath-Landsteiner syndrome:
(AKA Paroxysmal cold hemoglobinuria or Cold hemolysin IHA)
Mycoplasma pneumoniae,
measles
mumps, other flu-like viruses
DIC, TTP, SLE, malignant hypertension can manifest this type of anemia.
microangiopathic anemia.
RBCs phagocytized by macrophages in the spleen and liver describes what type of hemolysis?
extravascular
Vitamin B12 and/or folate deficiency can cause what type of anemia?
Megaloblastic anemia:
Major s/sx of SCA:
1) infarction
2) splenomegaly
3) predisposition to infection of encapsulated organ
4) chronic hemolysis
How does defective DNA synthesis as seen in megaloblastic anemias cause macrocytosis?
There is cell growth (G2) without cell division (M).
Explain how pernicious anemia can cause megaloblastic anemia.
Autoantibody to Intrinsic Factor or gastric parietal cells –> impaired or deficient IF –> B12 deficiency –> folate d
Autoantibody blocks attachment of B12 to IF. What kind of anemia?
Type I anti-IF pernicious anemia
PBS of hereditary spherocytosis reveals:
Spheroid RBC, loss of central pallor
Lab findings include: Hypersegmented neutrophils glossitis increased homocysteine normal methylmalonate
Folate deficient megaloblastic anemia
Lab findings include: Hypersegmented neutrophils glossitis increased homocysteine increased methylmalonate
B12 deficient megaloblastic anemia
Diphyllobothrium latum can cause what anemia?
B12 deficient megaloblastic anemia
What drugs can cause megaloblastic anemia?
methotrexate, trimethoprim (anti-folate drugs)
Lab findings:
Achlorhydria
(+) serum antibodies
atrophic glossitis
Most likely diagnosis:
pernicious anemia
Lab findings:
Macrocytic, hyperchromic RBC
Leukopenia with hypersegmented neutrophils
Thrombocytopenia
megaloblastic anemia
Lab findings of IDA?
Lab findings:
PBS = hypochromic microcytic
BM = decreased sideroblasts
Decreased iron and ferritin, increased TIBC
Triad of esophageal webs + atrophic glossitis + IDA = ?
Plummer Vinson Syndrome
Decreased serum iron
Increased serum ferritin
Decreased TIBC
Anemia of chronic disease
Decreased serum iron
Decreased serum ferritin
Increased TIBC
IDA
Increased serum iron
Increased serum ferritin
Decreased TIBC
Thalassemia
Failure or destruction of myeloid stem cells results in this type of anemia:
aplastic anemia
Hereditary aplastic anemia caused by DNA repair defect:
Fanconi’s anemia
A primary stem cell defect causing aplastic anemia can be described as:
idiopathic
Drugs which my cause failure or destruction of myeloid stem cells:
chloramphenicol, carbamazepine, phenytoin can cause aplastic anemia
Viral agents that can cause failure or destruction of myeloid stem cells:
CMV, parvovirus B19, EBV
Space occupying lesion that causes displacement and destruction of BM.
BM reveals SOL
PBS reveals pancytopenia and teardrop-shaped RBC
Myelophthisic anemia
Disease caused by deficiency of ADAMTS 13.
Thrombotic thrombocytopenic purpura.
In TTP, deficiency of ADAMTS 13 enzyme results in a decreased degradation of vWF multimers –> excessive activation/aggregation of platelets (thus thrombocytopenia) –> thrombosis
Secondary absolute polycythemia is caused by
increased EPO secretion
This syndrome is a stress polycythemia usually associated with hypertension, obesity, anxiety, smokers
Gaisböck syndrome
Mutation of this gene causes polycythemia vera
tyrosine kinase JAK2
Anemia presenting with cyanosis, pruritis, peptic ulcers
Polycythemia vera
Cyanosis - unoxygenated RBCs stay in low pressure circulation
Pruritis - increased basophils –> histamine
Peptic ulcer - increased basophil/histamine –> increased HCl secretion
This is a complication of polycythemia vera in which bone marrow no longer responds to elevated blood cells, which may develop into leukemia
myelofibrosis
Infections that may cause vessel wall abnormalities:
Meningococcemia, measles, rickettsia, endocarditis
Impaired formation of collagen which results in abnormal vessel walls may be caused by
scurvy
Ehlers Danlos syndrome
Drugs which can cause wall vessel abnormalities:
sulfas, penicillin (hypersensitivity)
Systemic hypersensitivity disease characterized by arthritis, purpura, and coliky abdominal pain.
Henoch–Schönlein purpura
Anemia with gait instability with decreased proprioception in lower extremities.
B12 deficient anemia
Autosomal dominant disorder characterized by dilated, tortuous BVs with thin walls that bleed readily:
hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)
Self-limited anti-platelet disorder in children following a VIRAL infection
acute idiopathic thrombocytopenic purpura
Anti-platelet autoimmune disease seen in adults (usually IgG class), especially reproductive females.
chronic idiopathic thrombocytopenic purpura
This disease is characterized by the pentad of: Microangiopathic hemolytic anemia FEVER transient NEURO deficits RENAL failure thrombocytopenia
Thrombotic thrombocytopenic purpura usually ocurs in the fourth decade of life
Primary absolute polycythemia is known as and is ____ independent
polycythemia vera
erythropoietin
(polycythemia vera is a myeloproliferative disorder with mutations that lead to growth of red cell progenitors independent of EPO)
Spontaneous bleeding in mucous membranes and excessive bleeding from wounds. Normal PC, elevated BT and possibly APTT.
von Willebrand’s disease
normal PC because platelets are unaffected
elevated PT because platelet plug formation is impaired (decreased vWF)
elevated or nomal APTT because Factor VIII is part of intrinsic pathway (vWF affects FVIII stabiliy)
Factor VII abnormality shows this test result
Prolonged PT (normal PC, BT, CT)
FVIII, IX, XI, XII abnormality shows this test result
Prolonged APTT (normal PC, BT, CT)
Deficiency of which clotting factor does not cause a bleeding disorder
F XII (Hageman)
Hemophilia A is caused by
F VIII deficiency
Hemophilia B is caused by
F IX deficiency
Bleeding disorder with prolonged PTT and normal PT. It is an X-linked recessive disorder.
Hemophilia (A or B)
Vit K disease results in deficiency of what factors?
II, VII, IX, X
Also seen in liver disease
Its pathology is based on hemolysis in the splenic capillaries due to the RBC’s abnormally sphere shape:
Hereditary spherocytosis
Widespread thrombosis causing fibrinolysis or consumption of clotting factors and platelets which ultimately results in bleeding is seen in what disorder?
disseminated intravascular coagulopathy
widespread thrombi then bleeding
This clinical syndrome associated with bleeding produces adrenal hemorrhaging because of fibrin thrombi within the microcirculation of the ADRENAL CORTEX
Waterhouse-Friderichsen syndrome
This clinical syndrome associated with bleeding causes pituitary necrosis after complicated delivery.
Sheehan’s postpartum necrosis.
Defective platelet adhesion - no GP Ib
Bernard Soulier
Defective platelet aggregation - no GP IIb/IIIa
Glanzmann Thrombasthenia
