HematologyJaynstein (Exam 1) Flashcards
T/F: Factor V Leiden is the most common inherited form of coagulopathy
true!
The liver produces all coagulation factors except factor ____
VIII
Hematocrit is the % of a sample of whole blood occupied by ______
intact RBC’s
If RDW is elevated, this suggests _______
large variability in sizes of RBCs
Where are common sites of blood loss?
GI tract
Urinary tract
Menses/menorrhagia
Blood donations
Complications of Thalassemia
- Hemochromatosis= iron overload
- Increased infection risk
- Bone deformities (secondary to bone marrow expansion)
What is the triad of sx for TTP?
Thrombocytopenic purpura
Microangiopathic hemolytic anemia
Neurological symptoms (seizure, AMS, TIA)
Von Willebrand Factor carries factor ______?
8
vWF carries factor VIII
How can you distinguish petechiae and purpura from a skin rash?
Petechiae, purpura, and ecchymoses do NOT blanch with pressure.
S/Sx of anemia
- Fatigue
- Tachycardia
- Hepatosplenomegaly
- Dyspnea on exertion
- Pallor
- Bone tenderness
2 steps used for describing anemia’s:
1) Describe based on RBC size using the MCV
2) Describe based on Hgb concentration with MCH and MCHC
Normal RDW range
11-15%
What is the normal range for platelets?
150k-450k/mL
Describe the process of antiphospholipid antibody syndrome.
An autoimmune disease in which people produce abnormal proteins (antiphospholipid antibodies)–>creates turbulent and stagnate blood flow–>thrombus forms
T/F? This patient should be admitted for IV antibiotics to treat the GI infection, which will fix the HUS.
False - antibiotics will cause lysis of bacteria releasing more toxins.
Normal Platelet count
100,000-450,000/mcL
List 3 causes of anemia
- RBC loss
- Decreased RBC production
- Increased RBC destruction
What is the pathophysiology of ITP?
IgG antibodies bind to platelets tagging them for destruction by the spleen
Describe lab findings for Thalassemia
- Mild to mod microcytic, hypochromic anemia.
- Normal iron studies
- NL to elevated reticulocyte count – bone marrow trying to compensate
- Blood smear shows target cells
- Hgb electrophoresis is definitive
Define hemarthroses
Bleeding into a joint capsule
T/F? An acceptable replacement anticoagulant is warfarin
False - replace with any other anticoagulant other than warfarin
Normal hemoglobin range for males?
13.5-16.5d/dl
Ferritin Lab:
- measures?
- normal range?
Stored iron, first lab to become low
- < 30mcg/dL in a pt with decreased H/H is always iron deficiency anemia
what is the hallmark lab value in polycythemia vera?
hematocrit >60%, but will see elevations in all cell lines.
Dx of APS?
-lab assessment for presence of antiphospholipid antibodies (aPL)
(3 possible lab tests exist and need positive results on at least 2 different ones to be able to dx)
Also: a positive test in the absence of a clot IS NOT a dx, just acknowledgement of risk
What are 3 main causes of thrombocytopenia?
think broad
Decreased platelet production
Increased platelet destruction
Other (sepsis or blood loss)
Majority of iron absorption occurs in the _______. Once absorbed, it’s transferred by _____
- duodenum
- transferrin
Blood smear findings of sickle cell anemia include which of the following?
a) Target cells
b) Sickled cells
c) Basophilic stippling
d) Howell-Jolly bodies
a, b, and d
Tx for HIT?
Stop heparin and replace with another anticoagulant
Avoid heparin indefinitely
Clinical findings for Thalassemia are _____ dependent
severity
**more severe cases can have pallor, splenomegaly, growth failure, bone deformities, jaundice
Using hematocrit and Hgb, define anemia in an adult female.
- Hematocrit: <37%
- Hgb <12 g/dL
What are the two main categories for normocytic anemia?
Hemorrhagic and non-hemorrhagic
Reticulocyte count= # of ______ RBC’s
immature
s/sx of polycythemia vera?
pruritis, HA, fatigue, splenomegaly
What is HIT?
Heparin Inducted Thrombocytopenia
T/F: IDA is life-threatening
False! rarely life-threatening
What is the triad of HUS?
Thrombocytopenia, renal failure, hemolytic anemia
What are some causes of decreased platelet production?
Bone marrow suppression/failure (medications, chemo, anaplastic anemia)
Bone marrow destruction (lysis, cancer)
Thalassemia Tx: major cases
- May require chronic blood transfusions, splenectomy, and bone marrow transplant
- Avoid iron supplements and Sulfonamides
**Only cure for major cases is allogeneic stem cell transplant
What is APS the underlying cause to?
- DVTs
- ischemic strokes
- miscarriages
What labs are likely to be low in Von Willebrand’s disease?
Plasma vWF and Factor VIII may be low
Describe Normocytic, Microcytic, and Macrocytic anemia using MCV
Normocytic= 80-100fl
Microcytic= <80 fl (decreased MCV)
Macrocytic= >100 fl (increased MCV)
MCH=
mean corpuscular hemoglobin (aka the average weight of Hgb per RBC)
What do you tell the parent of the child regarding the prognosis of idiopathic thrombocytopenic purpura?
The disease is self limiting and should resolve on its own
Hemochromatosis Tx
Chelation therapy= use of a chemical compound that combines with the metal poisoning for rapid and safe excretion
What is the treatment for G6PD?
avoiding known oxidative drugs
Thalassemia is defined as ______ _______ anemia
Microcytic hypochromic anemia.
Tx for folic acid deficiency?
Folic acid 1mg PO QD
The reticulocyte count comes back high, does this indicate hemolytic anemia or non-hemolytic anemia?
hemolytic anemia
What are your expected results on a blood smear of a patient with anaplastic anemia?
No abnormal cells
A child presents for evaluation of a rash, and easy bruising. He is thrombocytopenic, and further questioning reveals he recently got over a viral illness. What thrombocytopenia should you think of?
Idiopathic thrombocytopenic purpura
What is thrombocytopenia?
Low platelet count
What foods are rich in folic acid?
fruits and vegetables
Why is hemophilia more common in men?
Hemophilia is a sex linked hereditary gene that is located on the X chromosome.
Because men have only 1 X chromosome, they will have hemophilia if they acquire the gene on their only X chromosome.
Hemophilia A is due to a deficiency in factor _____,
while hemophilia B is due to a deficiency in factor ________.
VIII (8),
XI (9)
What is the treatment for an adult with ITP?
If platelets <20k or bleeding: Prednisone 1-2mg/kg/day IV immunoglobulin 1g/kg x 2 days Platelet transfusions as necessary Splenectomy is curative if chronic
Normal hematocrit range for females
36-44%
Examples of Microcytic anemias
- Iron Deficiency Anemia (IDA)
- Thalassemia
- Sideroblastic Anemia
What will happen to the Pt’s Hct level after starting Ferrous sulfate?
-hct level will be halfway to normal in 3 weeks, and within normal by 2 months!
**If hct is NOT normal in 2 months–> wrong dx, continued blood loss, or pt is non-compliant
-“May” d/c Ferrous sulfate 3-6 months after hct is WNL
_____ is the leading cause of anemia worldwide.
Iron deficiency anemia (IDA)
What is the treatment for all clotting disorders?
- Acute: heparin/LWMH, possible thrombectomy or thrombolysis
- Preventative: anticoagulants or ASA
- Avoid OPCs if possible
- Minimize risks: smoking cessation, manage co-morbidities (HTN, DM, HLD)
What is the most common congenital coagulopathy?
Von Willebrand’s Disease
Thalassemia is a hereditary disease of impaired ____ synthesis
Hgb
**Normal Hgbis comprised of 2α chains and 2β chains, those with Thalassemia have a defect in one of those chains
T/F? You can diagnose ITP if the patient is thrombocytopenic, has purpura, and recently had a viral infection.
False - you must exclude other causes of thrombocytopenia.