WEEK 1 Flashcards
After hematopoetic stem cells (HSCs), what are the two divisions of hematopoetic progenitor cells?
- myeloid progenitors - RBCs, platelets, WBCs (granulocytes, monocytes)
- lymphoid progenitors - B and T cells
What enzymes control erythropoeisis, thrombopoesis, and granulocyte production?
Erythropoetin, thrombopoietin, granulocyte colony stimulating factor (G-CSF)
How do EPO levels fluctuate?
- rise in response to hypoxemia
- rise in response to low hematocrit
- ## inhibited by IFN-gamma, TNF, inflammation can cause anemia
bone marrow failure
abnormal hematopoiesis due to bone marrow hypocellularity, which results in abnormal production of one or all blood cells
Severe aplastic anemia (SAA) criteria
- bone marrow hypocellularity
2. at least two cytopenias
idiopathic SAA
possibly immune mediated, no spontaneous recovery.
treatment:
- supportive care (transfusion, Abx)
- definitive therapy (HSC transplant, immunosuppressive therapy)
Fanconi anemia
INHERITED
- autosomal recessive DNA repair disorder
- abnormal thumbs, microophtlamia, cafe au lait spots
- predisposition to various cancers
- HALLMARK: CHROMOSOME BREAKAGE in response to DNA crosslinking agents
dyskeratosis congenita
INHERITED
- abnormal telomeres
- BMF develops by age 30
- mucocutaneous triad (abnormal skin pigmentation, oral leukoplakia, nail dystrophy
Diamond-Blackfan anemia
congenital red cell anemia (responds to steroids)
- macrocytic, hypoproductive anemia, increased eosinophils, congenital abnormalities
- bone marrow lacks erythroid precursors but otherwise normocellular
Acquired causes of BMF?
toxins, drugs, viruses
Parvovirus B19,
In acute intermittent porphyria there is a deficiency in what enzyme?
porphobilinogen deaminase which leads to increased porphyrins in blood and urine (presents with abdominal pain, tachycardia, HTN, neuropathy, psychosis…)
Porphyria cutanea tarda cause?
low levels of uroporphyrinogen decarboxylase (UROD)…can be inherited or acquired (via iron overload)…presents with skin/liver problems
striking feature: BULLOUS DERMATOSIS on light areas
In asplenic patients, what types of RBC forms would you see?
acanthocytes and Howell Jolly bodies
Prominent early portion of P>2.5
right atrial enlargement
delayed late portion of P with a bifid P in II
left atrial enlargement
RSR’ in leads V1/V2
right bundle branch block
RSR’ in leads V5/V6
left bundle branch block
long abnormal QT interval
toursades du pointes
long PR interval
drugs (digoxin, BB, CCB), ischemia or infarction, degeneration or calcification of AVN or His bundle
long QT
drugs, electrolytes (low K, Ca or Mg)
