Internal Med - Heme Flashcards
Acute lymphocytic leukemia (ALL) is MC in what age group
Children - MCC of malignancy peak age 3-7
Child + Lymphadenopathy + bone pain + bleeding + fever
ALL
Bone marrow with more than 20% blasts in a child with bone pain
ALL
60yo Male with fatigue, blood tests demonstrate severe anemia, decreased neutrophils, small, abnormal B lymphocysts with painless cervical lymphadenopathy and hepatosplenomegaly
CLL = Chronic lymphocytic leukemia
MC age for CLL
Middle age patient; Peak age of 50
DX of CLL on peripheral smear
SMUDGE cells on smear + mature lymphocytes
Tx of ALL and CLL
ALL = Highly responsive to chemo; Remission >90%
CLL = Tx with observation; if lymphocytes are >100,000 or symptomatic, tx with chemo
Acute Myelogenous leukemia MC in what age group
Most common in adults; 65 years
Dx of AML on peripheral smear
Auer Rods
Sx of AML
Anemia, DOE, dizziness; Splenomegaly may report early saiety; gum infiltration/gingivitis
Dx tests for AML
Bone marrow biopsy = definitive
CML dx
Philadelphia chromosome (Ph+); Bcr-Abl is the abnormal gene causing the switch of the philadelphia chromosome and the cell to grow out of control
Most have elevated lactate dehydrogenase level and elevated uric acid
AML on peripheral smear
Auer rods + blasts in an adult ot
Philadelphia chromosome
CML
Translocation of chromosome 9 & 22
Tx of AML and CML
AML → combination chemo, bone marrow transplant
CML → Gleevec (imantinib) which makes this a chronic disease state
Normocytic/normochromic or decreased MCV, decreased TIBC with normal or increased ferritin
Anemia of chronic dx
Dx of anemia of chronic dz
Requires presence of chronic infection, inflammation, or cancer; Microcytic or normocytic anemia
MCC of anemia of CKD
Chronic renal failure, anemia resulting from connective tissue disorders; HIV, SLE, Cancer, Cirrhosis, chronic infections
How does anemia of renal failure affect EPO
EPO is impaired d/t decrease in both EPO production + bone marrow responsiveness to EPO
Presence of renal insufficiency or failure + decreased EPO levels
Tx of anemia of CKD
Tx underlying disorder
Recombinant EPO and iron supplements
MC genetic bleeding disorder
Von Wille; Autosomal dominant
How to differentiate VwD from hemophilia
In VWD there is lack of hemarthrosism small amounts of superficial bleeding, common to have bleeding with a minor injury or petechiae
What factor affects VwD?
Decrease in VW factor and Factor 8
Tx of VwD
DDVAP Desmopressin or in excessive bleeding a transfusion concentrated blood clotting factors containing VW factor
What are the lab values associated with Hemophilia A & B
Increased PTT and normal plt count and functio
Most specific test for hemophilias
Functional assay for factor 8 & 9
Dx of VWD
CBC with plt count and coag studies → Normal CBC, normal plt count and increased bleeding time
Normal or prolonged aPTT and normal PT
Tx of hemophilia A & B
Replacement of factor 8 & 9; Pts should avoid situations that provoke bleeding; avoid blood thinners and anti platelet drugs
G6PD is a deficiency of
X linked enzyme defect
MC population G6PD is seen in
African Americans
Drugs that commonly cause hemolysis in pts with G6PD
Antimalarias
Analgesice
Sulnonamides
Dapsone
*Fava Beans
Oxidative stress (infection or drugs) + African American Male + Heinz bodies
G6PD
What is seen on a peripheral smear in G6PD
Heinz bodies and Bite cells on smear
MC procoagulant clotting factor
Factor V leiden
How does factor V cause hypercoagability
Mutated factor v resistant to breakdown by activated protein c → Hypercoag
Sx and PE findings in a pt with Factor V leiden
increased DVT and PE, especially in young patients
Lab findings in Factor V
Activated protein C assay → Factor v functional assay and confirm with DNA testing
Normal PT and PTT
Protein C deficiency
Autosomal dominant protein c mutation
Vitamin K dependent anticoag liver protein that stimulates fibronolysis and clot lysis
Dx of protein c deficiency
Protein C or S functional assay → Decreased protein c or s activity levels
What are pts with protein c deficiency at risk for?
Increased risk of skin necrosis while on warfarin