Hematology Flashcards
Fetal erythropoiesis occurs in: • Yolk sac (?-?) • Liver (?- ?) • Spleen (?- ?) • Bone marrow (?-?)
Fetal erythropoiesis occurs in:
• Yolk sac (3- 8 weeks) - yolk opens from 8-3pm
• Liver (6 weeks- birth) 6iver - living
• Spleen (10- 28 weeks) spleen in between
• Bone marrow (18 weeks to adult) 18 y.o.-adult
ABO classification
AB -?
O -?
AB -Universal recipient of RBCs; universal donor of plasma.
O - Receive any non-O -> hemolytic reaction.
Universal donor of RBCs; universal recipient of plasma.
Neutrophils - Specific granules contain:
Mafia bullies: “Break, Burn, take money and get lap dances.”
leukocyte alkaline phosphatase (LAP) -“Lap dances”
Collagenase - Break
Lysozyme - Burn
Lactoferrin - “take money (Iron)”
Azurophilic granules (lysosomes) contain: Break, break burn and take proteinases - break acid phosphatase - take myeloperoxidase - burn beta-glucuronidase break
Important neutrophil chemotactic agents:
P - platelet-activating factor. L - LTB4 I - IL-8 C - C5a K - kallikrein
Anisocytosis = ?
Poikilocytosis =?
Reticulocyte = ?
Anisocytosis = varying sizes.
Poikilocytosis =varying shapes.
Reticulocyte = immature RBC; reflects erythroidproIiferation.
Thrombocytes dense granules contain:
ADP, Ca2+
Thrombocytes alfa granules contain:
4 F’s:
vWF
fibrinogen
fibronectin
platelet factor 4
Eosinophils - Causes of eosinophilia
PACA-MAN:
Parasites Asthma Churg-Strauss syndrome Adrenal insufficiency (Chronic) Myeloproliferative disorders Allergic processes Neoplasia (eg, Hodgkin lymphoma)
Eosinophils - Produce
N - neurotoxin y M - major basic protein (MBP, a helminthotoxin) P - peroxidase H - histaminase i C - cationic protein
Basophils - Densely basophilic granules contain:
heparin (anticoagulant)
histamine (vasodilator)
Leukotrienes synthesized and released on demand.
Mast cells Activated by
Activated by TEC
T - tissue trauma
E - surface IgE cross-linking by antigen (IgE receptor aggregation)
C - C3a and C5a
Mast cells degranulation ->
release of histamine heparin tryptase eosinophil chemotactic factors.
Plasma cells Found in_________ and
normally do not______.
Plasma cells Found in the bone marrow and
normally do not circulate in peripheral blood.
Coagulation factors that require Ca+ and phospholipid
Activation of 7,9 -> 2
factor IIa activates
XIa, VIIIa, Va, XIIIa
factor XIII purpose
crosslinks fibrin
Anticoagulation pathways:
Antithrombin pathway (heparin-like molecule - enhances ATlll activity) Inhibits IIa, VIIa, IXa. Xa, XIa, Xlla
Protein C pathway - C->S
Inhibits Va, VIIIa
Shortest and Longest factor half lives
Seven - Shortest
Two - Tallest (longest)
Basophilic stippling
Aggregation of ribosomal precipitates.
Do not contain iron.
Heinz bodies
Hb denatures and precipitates
Howell-Jolly bodies
Basophilic nuclear remnants found in RBCs.
Pappenheimer bodies
Siderocytes containing basophilic granules of iron in sideroblastic anemias.
Ringed sideroblasts
Excess iron in mitochondria - Seen inside bone marrow smear with special staining
Microcytic anemia
Hemoglobin affected (TAIL): Defective globin chain: • Thalassemias Defective heme synthesis: •Anemia of chronic disease •lron deficiency •lead poisoning
Normocytic - Nonhemolytic anemia
- Iron deficiency (early)
- Anemia of chronic disease
- Aplastic anemia
- Chronic kidney disease
Normocytic - Hemolytic - Intrinsic anemia
INSIDE the cell you have (enzymes, Hb, membrane)
Membrane defects:
Hereditary spherocytosis
Paroxysmal nocturnal hemoglobinuria
Enzyme deficiencies:
G6PD deficiency
Pyruvate kinase deficiency
Hemoglobinopathies:
Sickle cell anemia
HbC disease
Normocytic - Hemolytic - Extrinsic anemia
- Autoimmune
- Microangiopathic
- Macroangiopathic
- Infections
Macrocytic - Megaloblastic anemia
DNA affected: Defective DNA synthesis •Folate deficiency •Vitamin B12 deficiency •Orotic aciduria
Defective DNA repair:
•fanconi anemia
Macrocytic - Nonmegaloblastic anemia
Diamond-Blackfan anemia
Liver disease
Alcoholism
Iron-deficiency anemia progression:
- dec ferritin. inc TIBC
- dec. serum iron, dec saturation.
- Normocytic anemia
- Microcytic anemia
Iron-deficiency anemia Causes:
Losing - chronic bleeding
Not getting - malnutrition, absorption disorders, GI surgery
Need more - inc demand (eg, pregnancy)
alfa thalassemia types
a -thalassemia minima
a -thalassemia minor
Hemoglobin H disease (HbH);
Hemoglobin Barts disease;
Beta -thalassemia types
beta - thalassemia minor - HbA2 (> 3.5%) on electrophoresis.
beta - thalassemia major
HbS/beta-thalassemia heterozygote
beta-thalassemia major results in
H - hemochromatosis (2° to blood transfusion)
E - Extramedullary hematopoiesis -> hepatosplenomegaly.
P - Parvovirus Bl9-induced aplastic crisis risk.
A - anemia (severe microcytic, hypochromic)
T - target cells
o
M - Marrow expansion: “crew cut” skull x-ray, “chipmunk” facies
A - anisopoikilocytos (increased)
Sideroblastic anemia causes
R - reversible
A - acquired (myelodysplastic syndromes)
G - genetic (eg, X-linked defect in ALA synthase gene),
Reversible Sideroblastic anemia causes
C - copper deficiency
A - alcohol, is the most common
L - lead
B - B6 deficiency
D - drugs: isoniazid, linezolid
o
g
Symptoms of LEAD poisoning:
- Lead Lines on gingivae (Burton lines) and on metaphyses of long bones on x-ray.
- Encephalopathy and Erythrocyte basophilic stippling.
- Abdominal colic and sideroblastic Anemia.
- Drops- wrist and foot drop.
Anemia of chronic disease associations
A - autoimmune diseases (eg, SLE, rheumatoid arthritis).
I - infections
N - neoplastic disorders
K - kidney disease
Aplastic anemia
Fan DRIVe
F - Fanconi anemia
D - drugs (ABC eg, alkylating agents/antimetabolites, benzene, chloramphenicol)
R - Radiation
I - Idiopathic (immune-mediated, primary stem cell defect)
V - Viral agents (EBV, HIV, hepatitis viruses)
e
Fanconi anemia vs. Diamond-Blackfan anemia
Both have Short stature and limb defects.
Diamond-Blackfan anemia - defect in erythroid progenitor cells.
Fanconi anemia - DNA repair defect causing bone marrow failure.
Fanconi anemia also presents with tumors/ leukemia, cafe-au-lait spots