Hematology Flashcards
Etiology of hemophilia A?
Deficiency in factor VIII
Etiology of hemophilia B?
Deficiency in factor IX
Change to PTT in hemophilia A and B?
Prolonged
Change to PT in hemophilia A and B?
Normal
Inheritance pattern of hemophilia A and B?
X-linked recessive
Clinical presentation for hemophilia A and B?
Joint hemorrhage; No excessive bleeding after minor cuts
Treatment of hemophilia A?
Human factor VIII concentrate
Treatment of hemophilia B?
Human factor IX concentrate
Treatment for mild cases of hemophilia A?
Desmopressin
Mechanism of action of desmopressin in treatment of hemophilia A?
Desmopressin causes release of VWF and factor VIII from endothelial tissues
Inheritance pattern of hemophilia C?
AR
Etiology of hemophilia C?
Factor 11 deficiency
Best treatment for hemophilia C?
Fresh frozen plasma
Change to PTT in hemophilia C?
Prolonged
Change to PT in hemophilia C?
Normal
Clinical presentation factor 12 deficiency?
No problem with bleeding (can undergo surgery without worry of excessive bleeding)
Change to PTT in factor 12 deficiency?
Extremely prolonged
Change to PT in factor 12 deficiency?
Normal
Inheritance pattern of factor 13 deficiency?
AR
Lab values seen in factor 13 deficiency?
Normal PT and PTT
Diagnostic test for factor 13 deficiency?
Urease clot solubility test
Best treatment for factor 13 deficiency?
Small amounts of fresh frozen plasma every 3–4 weeks
Condition associated with factor X deficiency?
Amyloidosis
Most common hypercoagulable hereditary state?
Factor V Leiden
Etiology of Factor V Leiden?
Activated Protein C resistance
Best treatment for Factor V Leiden?
Indefinite warfarin
Medication that is contraindicated in Factor V Leiden?
OCPs
3 natural anti-coagulants that may cause thrombotic disorders if deficient?
Protein C, Protein S, Anti-thrombin III
33 yo female with known history of SLE; no history of miscarriage or abnormal arterial or venous clotting - what is next best step for treatment?
Observe
What type of thrombosis is associated with homocystinuria?
Both venous and arterial
Primary hemostasis is mediated by …
Platelets
Secondary hemostasis is mediated by …
Coagulation factors
Etiology of ITP?
IgG directed against GPIIb/IIIa on platelet surface
Coomb’s test for ITP?
(+)
Lab change associated with ITP?
Thrombocytopenia
Clinical presentation of ITP?
Thrombocytopenia in adolescents following viral URI
Diagnostic test required for ITP in asymptomatic patient with isolate thrombocytopenia?
Review peripheral blood smear … no further workup required
Best treatment for ITP in asymptomatic patient?
Supportive care
Best treatment for ITP in symptomatic patient?
Corticosteroids
5 aspects of clinical presentation associated with TTP?
FTKCS … Fever, thrombocytopenia, Kidney failure, Confusion, schistocytes
Change to PT and PTT in TTP?
NML
Change to LDH in TTP?
Elevated … due to RBC hemolysis
Medication associated with TTP?
Ticlopidine … grandfather of clopidogrel
Additional AE of Ticlopidine?
Agranulocytosis
Best treatment for TTP?
Plasmapheresis (plasma exchange)
Best treatment for TTP in patients in which plasmapheresis is not available?
FFP
Coomb’s test for TTP?
(-)
Change to PT and PTT in HUS?
NML
3 aspects of clinical presentation for HELLP syndrome?
Hemolytic anemia, Elevated LFTs, Low Platelets
Appearance of blood smear in HELLP syndrome?
Schistocytes
Change to PT and PTT in HELLP?
NML
Major complication of HIT?
Thrombosis … (not bleeding)
Best treatment for HIT?
Stop heparin … begin Argatroban
MOA of Argatroban?
Direct thrombin inhibitor
Inheritance pattern of Von Willebrand Disease?
AD
Lab change seen in setting of Von Willebrand Disease?
Prolonged bleeding time, Prolonged PTT
2 functions of Von Willebrand Factor?
Adhesion of platelets; Carrier protein for Factor VIII
Definitive diagnosis for Von Willebrand Disease?
Von Willebrand Factor assay
Best treatment for mild cases of Von Willebrand Disease?
DDVAP (vasopressin)
MOA of DDVAP (vasopressin) in treatment of Von Willebrand Disease?
Enhances release of VWF and Factor VIII from endothelial cells
Etiology of Bernard-Soulier Syndrome?
Deficiency in GPIb
Lab change associated with Bernard-Soulier Syndrome?
Low platelet count
Etiology of Glanzman Thrombasthenia?
Deficiency in GPIIb/IIIa
Glanzman Thrombasthenia mimics the effects of which drug?
Abciximab
Change to platelet count in setting of Glanzman Thrombasthenia?
NML
Inheritance pattern of Glanzman Thrombasthenia?
AR
Inheritance pattern of Bernard-Soulier Syndrome?
AR
Change to PT and PTT in DIC?
Prolonged
Coomb’s test for HUS?
(-)
Coomb’s test for HELLP?
(-)
Coomb’s test for DIC?
(-)
Coomb’s test for Microangiopathic Anemia?
(-) … RBC fragmentation is mechanical … (not immunologic)
4 conditions that feature schistocytes?
HUS, TTP, HELLP, DIC
Alternate name for acanthocytes?
Spur cells
Condition associated with acanthocytes?
Liver disease, Abetalipoproteinemia
Inheritance pattern of Abetalipoproteinemia?
AR
Etiology of Abetalipoproteinemia?
Lipid malabsorption by enterocytes
Clinical presentation of Abetalipoproteinemia?
Diarrhea
Condition associated with burr cells?
Kidney disease
Which drug may cause sideroblastic anemia?
Isoniazid
Connection between ETOH abuse + Isoniazid and sideroblastic anemia?
Both decrease Vitamin B6
Patient presents with sideroblastic anemia on blood smear; Denies HX of ETOH use and Isoniazid therapy – next step of workup?
Check lead levels, erythrocyte protoporphyrin
___ refers to deposition of lead sulfide along teeth and gingiva, seen in setting of lead poisoning
Burton’s line
Where does lead typically deposit in bones?
Metaphysis
Condition associated with teardrop-shaped RBCs?
Myelofibrosis
Alternate name for RBCs that are teardrop-shaped?
Pyriform
Pathogen that is teardrop-shaped?
Trichophyton rubrum
Classic cell type seen in Myelodysplasia?
Pseudo-Pelger-Huet
Description of Pseudo-Pelger-Huet cells?
Bilobed PMNs
Conditions associated with Target cells?
Hemoglobinopathies, asplenia
Inheritance pattern of G6PD deficiency?
X-linked recessive
Etiology of bite cells in G6PD deficiency?
Attempt of spleen to remove Heinz bodies
Change to serum iron in iron deficiency anemia?
Low
Change to TIBC in iron deficiency anemia?
High
Change to transferrin saturation in iron deficiency anemia?
Low
Change to ferritin in iron deficiency anemia?
Low
Change to transferrin receptors in iron deficiency anemia?
High
Change to serum iron in anemia of chronic disease?
Low
Change to TIBC in anemia of chronic disease?
Low
Change to transferrin saturation in anemia of chronic disease?
Low
Change to ferritin in anemia of chronic disease?
High
Change to transferrin receptors in anemia of chronic disease?
NML
Most specific method of diagnosing iron deficiency anemia?
Bone marrow iron stain
Next step of workup for iron deficiency anemia, if colonoscopy is (-)?
EGD
Most common pathogen responsible for iron deficiency anemia?
Ancylostoma duodenale … (hookworm)
Alternate name for Osler-Weber-Rendu Syndrome?
Hereditary Hemorrhagic Telangiectasia
Inheritance pattern of Hereditary Hemorrhagic Telangiectasia?
AD
1st step of treatment for iron deficiency anemia?
Oral iron supply
2nd step of treatment for iron deficiency anemia?
IV iron
Inheritance pattern of hereditary sideroblastic anemia?
X-linked
Deficiency associated with sideroblastic anemia?
Vitamin B6
2 medications associated with sideroblastic anemia?
Isoniazid, Chloramphenicol
Renal disorder associated with lead poisoning?
Chronic interstitial nephritis with papillary necrosis; Type 2 RTA
How can you distinguish between b thalassemia minor and a thalassemia trait?
b thalassemia minor = increased HbA2, HbF; a thalassemia trait = NML HBG electrophoresis
2 types of normocytic anemia?
Anemia of chronic disease, Aplastic anemia
2 conditions associated with Pure RBC Aplasia?
Parvo B19 infection, Thymoma
Only type of inherited aplastic anemia?
Fanconi Anemia
Inheritance pattern of Fanconi Anemia?
AR
Portion of nephron that is affected by Fanconi Syndrome?
Proximal tubule
Are transfusions from family members preferred or avoided for patients with aplastic anemia?
Avoided
Why should transfusions from family members be avoided in patients with aplastic anemia?
Sensitize the patient to HLA antigens
2 pathogens associated with Plastic Anemia?
Hepatitis B, Hepatitis C
What prevents complement-mediated destruction of RBCs in Paroxysmal Nocturnal Hemoglobinuria?
Loss of Glycosyl phosphatidyl inositol (GPI) anchor protein
Most common cause of death in Paroxysmal Nocturnal Hemoglobinuria (PNH)?
Venous thromboembolism (DVT, hepatic vein thrombosis)
Hematologic malignancy associated with Paroxysmal Nocturnal Hemoglobinuria?
AML
3 treatments of choice for Paroxysmal Nocturnal Hemoglobinuria?
Warfarin (protection against venous thromboembolism), Corticosteroids, Iron supplementation
Which DOC in Paroxysmal Nocturnal Hemoglobinuria targets CD5 to minimize hemolysis?
Eculizumab
AE of Eculizumab?
Increased risk of meningococcal infection
3 possible signs of hemolysis on blood smear?
Schistocytes, Sickle cells, Spherocytes
Change to LDH in setting of hemolytic anemia?
Increased
Change to reticulocytes in setting of hemolytic anemia?
Increased
Change to Haptoglobin in setting of hemolytic anemia?
Decreased
Change to Indirect bilirubin in setting of hemolytic anemia?
Increased
Most common cause of death in patients with Sickle Cell Anemia?
Acute chest syndrome
Most common renal manifestation of Sickle Cell Anemia?
Isosthenuria … inability to concentrate urine
When is treatment for Sickle Cell Anemia with hydroxyurea contraindicated?
Pregnant patients … treat with oxycodone + acetaminophen
Best treatment for patients with Sickle Cell Anemia who develop aplastic anemia?
Folic acid supplementation
MSK complication of Sickle Cell Anemia?
Femoral head osteonecrosis
Best treatment for Sickle Cell patients with priapism?
Blood exchange transfusion
Best treatment for otherwise healthy patients with priapism?
Aspiration of blood from corpus cavernosum + Pseudoephrine
Coomb’s Test in Hereditary Spherocytosis?
(-)
Alternate name for spherocytes in Hereditary Spherocytosis?
Polychromatophilic RBCs
Best treatment for symptomatic patients with Hereditary Spherocytosis?
Splenectomy
Best test for patient with suspected Autoimmune Hemolytic Anemia (AIHA)?
Coomb’s Test … (direct antiglobulin test)
Location of Ig in (+) Direct Coomb’s Test?
On RBC membrane
Location of Ig in (+) Indirect Coomb’s Test?
In serum
Ig associated with warm AIHA?
IgG
Warm AIHA is ___vascular
Extra
Warm AIHA is intra___
Splenic
2 conditions associated with Warm AIHA?
SLE, Lymphoma
Best treatment for Warm AIHA?
Corticosteroids + Splenectomy
Ig associated with cold AIHA?
IgM
Cold AIHA is ___vascular
Intra
Cold AIHA is intra___
Hepatic
4 conditions associated with Cold AIHA?
Malignancy, Mono, Mycoplasma, PNH
Which type of AIHA is complement-mediated?
Cold
