Flashcards in Heme/Onc Deck (48):
Surface receptor for macrophage
CD14 - this is the LPS receptor
Causes of eosinophilia
NAACP- Neoplasia, Asthma, Allergies, Connective tissue disease, Parasites
If you see Basophilia
AB blood group
Universal blood receiver, universal donor of plasma
O blood group
Universal donor of blood, universal receiver of plasma
2, 7, 9, 10, 11, 12. Main function is to inactivate IIa and Xa
Test for VWF disease
decreased agglutination on Ristocetin assay, ristocetin activates VWf to bind to Gp1b
Defect in GpIb
Defect in GpIIb/IIIa- works like abciximab
Reasons for Basophillic stippling
LATA (Latte)- Lead poisoning, Alcohol, Thalassemia, Anemia of chronic disease
Reasons for Target cells
HALT- Hemoglobin C disease, Asplenia, Liver disease, Thalassemia
Plummer Vinison syndrome
Iron deficiency anemia, esophageal webs, atrophic
No a globin, leads to hydrops fetalis. Hb Barts is a gamma4 formation. (Common in asia)
Three alpha globin gene deletion. Leads to beta4 conformation, called hemoglobin H.
alpha2gamma2 (Think of a baby in a crib, the gamma sign looks like a crib? Maybe?)
Cause of beta thalassemia
point mutations in splice sites and promoters, defect in alternative splicing.
Inhibits ferrochelatase and ALA dehydratase. LEAD- Lead lines on gingivae and long bones of x-ray (burton lines), Encephalopathy and erythrocyte basophilic stippling, Abdominal colic or Sideroblastic Anemia, Wrist and foot Drop, Dimercaprol, EDTA, Succimer used for chelation.
Causes of sideroblastic anemia
X-Linked defect in DALA sythase (RLS of Heme synthesis). Lead poisoning. Alcohol (MOST COMMON). Pyridoxine deficiency.
Defect in pyrimidine synthesis (UMP synthesis). WILL NOT SEE HYPERAMMONEMIA like you will in OTC deficiency. Give Uridine Monophosphate.
Signs of intravascular hemolysis
Increased LDH, decreased haptoglobin, increased hemoglobinuria
Signs of extravascular hemolysis
Increase in unconjugated hyperbilirubinemia (Jaundice). Increased LDH
Osmotic fragility test +. Can do Eosin-5-Maleimide test for screening. Increased MCHC, extravascular hemolysis.
Paroxysmal Nocturnal Hemoglobinuria
No GPI anchor to decay accelerating factor to stop complement from lysis. CD55/59 negative. Coombs negative hemolysis, pancytopenia, venous thrombosis. treat with Eculizumab
Auto Immune Hemolytic Anemia with warm agglutinin
IgG Ab. Seen in SLE, CLL, Methyldopa treatment
IgM. Seen in Mononucleosis and Mycoplasma Pneumo (All M's)
Acute intermittent porphyria
No porphobilinogen deaminase. Acumulation of porphobilinogen, DALA, coporphobilinogen (urine). Symptoms : 5P’S- PAINFUL ABDOMEN, PORT WINE COLORED URINE, POLYNEUROPATHY, PSYCH DISTURBANCES, PRECIPITATION BY DRUGS, ALC, STARVATION Treat with glucose and heme, inhibiting ALA synthase
Porphyria Cutanea Tarda
Defect in Uroporphyrinogen Decarboxylase. Tea colored urine, blistering cutaneous photosensitivity.
Hemarthrosis, easy bruising points to...
Mucosal bleeding, epistaxis, petichiae points to...
Ab against GpIIb/IIIa
Defect in ADAMTS13. Large VWf multimers lead to easy clotting in microvasculature but doesn't use up coag factors so you're not getting DIC. Neuro and renal symptoms, thrombocytopenia, microangiopathic anemia. Treat with exchange transfusion and steroids.
Treatment for VWf disease
DDAVP (increased VWf release)
Can ATIII deficiency be cause by nephritic syndome?
No. But it can with a nephrotic syndrome.
Blood transfusions can lead to...
Hyperkalemia (due to blood cell lysis in storage), Hypocalcemia (blood is stored with citrate, a Ca2+ chelator), Iron overload.
Difference between leukemoid reaction and CML
Leukemoid reaction has high Leukocyte ALP, low in CML.
50% of Hodgkin lymphoma are associated with this virus
What gene is on chromosome 14 that creates all these lymphomas when a different gene is translocated to its spot?
Ig heavy chain (Highly active in B cells)
Mantle Cell lymphoma
CD5+. 11:14 translocation. Cyclin-D overexpressed.
14:18 translocation. BCL2 overexpressed (prevents apoptosis through mitochondrial stabilization). Can see waxing and waning lymphoma.
Bilobed PMN with single strand of chromatin connecting the lobes. Seen in chemo treatment
Leukemia in someone <15
Leukemia in middle aged person
AML or CML (Goljan)
Leukemia in old person
Hairy cell leukemia
TRAP+. Dry tap bone marrow. Treat with cladribine which inhibits Adenosine deaminase.
Langerhans Cell histocytosis
Presents in CHILD as rash, lytic bone lesions, recurrent otitis media with MASS OF MASTOID BONE. CD1a+, S100+ (mesodermal origin). Tennis racket birbeck granules.
non-receptor tyrosine kinase