HEME 4 Flashcards
What is the main difference between B-Thalassemia Major and Minor?
B-Thalassemia Minor = Decreased production of B gene
- -> usually asymptomatic
- -> Mild hypochomic microcytic anemia
B-Thalassemia Major = NO production of B gene
–> Severe anemia
In B-thalassemia minor, because you have decrease in B globulin production, what other types of hemoglobin do you see a compensatory increase in?
Increase in: HbgA2 (2 alpha, 2 delta) Hbg F (2 alpha, 2 gamma)
Decrease in: Hgb A (2 alpha, 2 beta)
In B thalassemia Major, you have no beta globulin so you are in need of constant what to sustain life?
How do you dx B thalassemia Major?
Frequent transfusions
DX = Hbg Electrophoresis
What do you see on Hbg electrophoresis in:
- Alpha thalassemia minor?
- Alpha thalassemia major?
- B- thalassemia minor?
- B- thalassemia major?
- Alpha thalassemia minor:
- normal - Alpha thalassemia major
- Increase in HgH band - B- thalassemia minor
- Increase in HbA2 band
- Increase in HbF band - B- thalassemia major
- Increase in HbA2 band
- Increase in HbF band
What is the inheritance pattern of Thalassemias?
Autosomal recessive
Immune thombocytopenia presents as an ISOLATED thrombocytopenia, with labs showing no other abnormality. It is commonly a post viral phenomenon that is idiopathic….
What can you see on Blood smear?
TRX?
Megakaryocytes (platelet precursors, that are 20-30X size of RBC with lots of cytoplasm)
TRX:
1. Skin manifestation only –> Observation
- Bleeding OR Plt < 30k –> IVIG or Steroids
What is the pathophysiology of Thrombotic Thrombocytopenic Purport?
What are the constellation of symptoms seen in Thrombotic Thrombocytopenic Purpura? (TTP)
Do you see any changes in PT or PTT in TTP?
TRX?
Autoimmune Abs generated against ADAMS13, a plasma metalloprotease, responsible for cleaving vWF from endothelial cells….Decreased ADAMS13 –> Increased microthombi formation as platelets aggregate on vWF.
HUS + fever + AMS (clots in brain)
HUS=
- MAHA
- Renal failure
- Thrombocytopenia
PT and PTT normal because no consumption of clotting factors. Micro thrombi form throughout the body, causing sharing of RBC.
TRX= Urgent Plasmapharesis (to remove auto Ab)
What do the following measurement reflect?
PT (INR)? What medication increased PT?
PTT? What medication increases PTT?
Thrombin time?
Bleed time?
PT: Extrinsic Pathway (Warfarin)
PTT: Intrinsic Pathway (Heparin)
Thrombin time: Inversely related to Fibrinogen { }
Bleed time: Platelets
What is DIC?
Labs show?
Abnormal activation of coagulation sequence that results in formation of micro thrombi all over the body. You end up consuming Plt, Fibrin, and all coagulation factors.
LABS:
- Low platelets
- Schisteocytes
- Decreased Fibrinogen
- Increased PT, PTT, TT, bleeding time.
- Increased D-Dimer
HIT occurs how many days after starting Heparin?
5-10 days.
What types of cardiac problems can you get with Radiation therapy to chest?
What type of cardiac problems do you typically see with cardiotoxic chemotherapy (Anthacyclines)
Radiation therapy:
- Heart block
- Pericarditis
- Cardiac fibrosis w restrictive cardiomyopathy
- Increase in MI/CAD
- Valvular Regurg (MR, AR)
Anthracycline:
- Dilated cardiomyopathy and low EF.
In am emergent bleed, for a patient on Warfarin, you need to give IV Vitamin K to reverse Warfarin’s action and ALSO give either Prothrombin Complex Concentrate or Fresh Frozen Plasma (both which replete vitamin K dependent clotting factors)…
How do you choose PCC vs FPP
In an emergent situation where you need to reverse Warfarin in minutes, use PCC…
FPP requires late volume to administer (>2 L) and also have to be matched for compatibility (all of which takes time) .
What medication can you use to reverse platelet dysfunction, 2/2 to Chronic Kidney Disease, during an acute bleed?
What is the mechanism of action?
IV Desmopressin
MOA: increase release of Factor 8 (vWF) from epithelium.
A patient had a DVT 1 month ago and has been on Warfarin therapy after discharge from hospital.
What is the next step in management if the patient comes to the clinic with expanding or worsening DVT due to sub therapeutic INR?
Admit to hospital and START HEPARIN, while Warfarin is adjusted.
A patient is recovering from mononucleosis, but is experiencing continued lymphadenopathy for 4 weeks beyond complete resolution of other symptoms…
What should you do next in management?
BX lymph nodes to rule out malignancy if lymphadenopathy lasting more than 3-4 weeks after resolution of mono symptoms.
