Hematology Flashcards
List three ways to describe the genetic abnormality in CML?
922 - BCR-ABL gene - Philadelphia chromosome
What leukemia would lymphoblast be associated with?
ALL (Blasts = Acute)
What leukemia would myeloblasts be associated with?
AML (Blasts = Acute)
What gene mutation is associated with polycythemia vera?
JAK2 mutation
TTP symptom Mnemonic: FAT-RN. What dose FAT-RN stand for?
Fever - Anemia - Thrombocytopenia - Renal failure - Neurologic involvement
List two conditions which predispose a patient to antiphospholipid syndrome.
Systemic Lupus Erythematosus - Women with recurrent abortions
What disorder is a hemoglobin electrophoresis result of “SA” indicative of?
Sickle cell trait
What disorder is a hemoglobin electrophoresis result of “SS” indicative of?
Sickle cell disease
What type of anemia are cephalosporins PCN NSAIDS methyldopa associated with?
Autoimmune hemolytic anemia
Erythropoietin analogs should be suspended once the hemoglobin is >_____?
11 mg/dl
Erythropoietin analogs should only be initiated if the hemoglobin is < ____?
10 mg/dl
What two types of anemia have macroovalocytes?
B12 and folate deficiency
Which anemia has a decreased MCV, decreased MCH (hypochromic) yet normal TIBC and ferritin?
Thalassemia
Acute chest syndrome occurs during the acute phase of which anemia?
Hemolytic sickle cell crisis
What are some common oxidative events which may trigger hemolysis in G6PD deficiency?
Antimalarials - Sulfa drugs - Aspirin - Febrile Illness - Acidosis - Fava beans
What type of anemia does a + osmotic fragility test indicate?
Hereditary Spherocytosis
What do an increased LDH, decreased Haptoglobin and an increased indirect Bilirubin represent?
Hemolysis
What type of anemia does a + direct coombs indicate?
Autoimmune hemolytic anemia
What does a > 50% reduction in platelet count within 7-10 days of exposure to heparin define?
Heparin Induced Thrombocytopenia (HIT) syndrome
Which type of anemia is most likely with associated leukopenia and thrombocytopenia?
Aplastic anemia
How would you expect TIBC and ferritin to present in anemia of chronic disease?
Decreased TIBC - Normal/increased Ferritin
Anemia of renal failure is defined by decreased levels of which hormone?
Erythropoietin
Can oral B12 be used as an initial treatment in a B12 deficient patient?
Yes - Oral B12 is considered initial therapy - Other routes if oral fails or pernicious anemia is diagnosed
Which route is best for administering B12 to a deficient patient?
Sublingual IM or deep subcutaneous
What tests other than a Schilling test are used to diagnose pernicious anemia?
Increased Methylmalonic acid levels - + Intrinsic factor and parietal cell antibodies
Increased MCV with smooth tongue, loss of proprioception and vibratory sense is likely which type of anemia?
B12 deficiency
List 5 common causes of folate deficiency.
EtOH abuse - Advancing age - Gastric bypass - Pregnancy - Methotrexate
What symptom differentiates B12 from folate deficiency ?
Neuropathy occurs with B12 deficiency but not with folate deficiency
Slowly decreasing MCV in an elderly patient?
Occult GI blood loss
Pica is a specific symptom of which type of anemia?
Iron deficiency anemia
Decreased Ferritin and Increased TIBC?
Iron deficiency anemia
What surgery can induce B12 deficiency?
Gastric bypass
Heavy menstrual cycle gum bleeding with teeth brushing?
Von Willebrand deficiency
What tests will confirm your diagnosis of multiple myeloma?
Serum: Protein electrophoresis may show monoclonal proteins - Urine: Bence Jones proteins
Autoimmune bleeding disorder in which patients develop antibodies against their own platelets?
Idiopathic Thrombocytopenia Purpura (ITP)
Deficient factor in hemophilia B?
Factor IX
Deficient factor in hemophilia A?
Factor VIII
What type of leukemia is most commonly seen in older men?
Chronic lymphocytic leukemia (CLL)
What is the most common type of kids leukemia?
Acute lymphocytic leukemia (ALL)
What does the presence of Heinz bodies indicate?
G6PD deficiency (May also be found in thalassemia)
Anemia due to renal failure may be confirmed by which test?
Decreased Serum erythropoietin levels
What type of megaloblastic anemia causes neurologic deficits?
Vitamin B12 deficiency
What type of anemia can occur in hypothyroidism?
Macrocytic non-megaloblastic anemia
What factor is produced in the stomach and required for Vitamin B12 absorption?
Intrinsic factor
What test confirms the diagnosis of pernicious anemia?
Schilling test or + antibodies to intrinsic factor and parietal cells
Which anemia is associated with bilirubin cholelithiasis?
Hereditary spherocytosis
What is the best test to determine whether a patient as a hemolytic anemia?
Reticulocyte count and haptoglobin
Which anemia has all 3 cells lines affected: Anemia leukopenia and thrombocytopenia?
Aplastic anemia
What is the Dx? Very low MCV, normal/high RBC, normal RDW and high/normal serum iron normal TIBC.
Thalassemia
What is the Dx? Low MCV, low RBC, high RDW, low serum iron, high TIBC.
Iron Deficiency Anemia
What is the Dx? Microcytic hypochromic anemia, low serum iron and low TIBC. Unresponsive to iron therapy.
Anemia of chronic disease
What is the result of homozygous alpha thalassemia (Alpha major) genetics?
Fetal death
Basophilic stippling of RBCs?
Lead poisoning (May also occur in thalassemia and chronic EtOH abuse)
Type of anemia in which serum iron is decreased and TIBC is increased?
Iron deficiency anemia
What is the Dx? Teenage African American with severe bone and joint pain severe anemia.
Sickle cell disease
What is the etiology of pernicious anemia?
Antibodies to gastric parietal cells and intrinsic factor
Increased serum protein with a decreased albumin/globulin ratio?
Multiple myeloma
Reed Sternberg cells?
Hodgkins lymphoma
Philadelphia (Ph) chromosome?
Chronic myeloid leukemia (CML)
Auer rods?
Acute myeloblastic leukemia (AML)
What hematological malignancy has a bimodal incidence by age of 15-45 years old or >60 years old?
Hodgkin lymphoma
Which malignancy has a peak incidence of 20-40 years old and can be associated with HIV?
Non-Hodgkin Lymphoma
Painless lymphadenopathy indicative of which malignant disorder?
Hodgkin lymphoma
What are the organ systems which may be involved in Thrombotic thrombocytopenia purpura (TTP)?
Renal - Neuro - Cardiac
List the four lab findings which support a diagnosis of DIC.
Decreased Fibrinogen - Thrombocytopenia - Increased D-Dimer - Increased PT and PTT
What disorder should be considered for a patient on warfarin who develops diffuse necrotic skin lesions?
Protein C deficiency
How is factor V Leiden disorder confirmed?
Activated protein C resistance assay - Genetic testing definitive
What is the most common genetic hypercoagulable state?
Factor V Leiden
List the three tests to confirm an DX of antiphospholipid syndrome in a hypercoagulable patient?
Lupus anticoagulant - Anticardiolipin antibody ELISA - Anti-beta2-glycoprotein-I ELISA
List the four direct thrombin inhibitors which may be used in HIT.
Lepirudin - Bivalirudin - Argatroban - Fondaparinux
Which antibody is positive in HIT syndrome?
+ platelet factor 4 (PF4) antibody
Which food is known to trigger hemolysis in a patient with G6PD deficiency?
Fava beans
List three medications which may trigger hemolysis in a patient with G6PD deficiency.
Antimalarials - Sulfa drugs - Aspirin
Which groups are most likely to be affected by G6PD deficiency?
Black or Mediterranean males
List three medications which may trigger autoimmune anemia.
Cephalosporins - Penicillins - NSAIDs
What is the most common autoimmune disorder associated with hemolytic anemia?
Systemic lupus erythematosus (SLE)
Which anemia is most commonly associated with EtOH abuse?
Folate deficiency
Priapism may occur in which acute anemia?
Sickle cell crisis
What test is diagnostic for sickle cell disease?
Hemoglobin electrophoresis
Which test is more sensitive than B12 levels for B12 deficiency in early disease?
Increased Methylmalonic acid levels occur in early B12 loss and may precede decreased serum B12 levels
What is the chronic treatment of Von Willebrand disease?
Chronic treatment: Factor VIII replacement
What is the acute treatment of Von Willebrand disease?
Acute/initial treatment: DDAVP
What assay is definitive for Von Willebrand disease?
Ristocetin cofactor assay
What factors are affected in Von Willebrand disease?
Von Willebrand factor and factor VIII are both decreased
What is first line treatment for Idiopathic thrombocytopenia purpura?
Steroids
Christmas disease is another name for deficiency of which clotting factor?
Decreased Factor IX (AKA Hemophilia B)
What disorder is usually diagnosed early in childhood and has hemarthrosis bruising and bleeding?
Hemophilia
How is hemophilia inherited?
X-linked recessive
What is the Dx? 75 year old male with adenopathy WBC 150K (90% lymphocytes).
CLL
What is the Dx? Child with fever bleeding bone pain and adenopathy?
ALL
What are the two medications available for treatment of anemia due to renal failure?
epoetin alfa and darbepoetin alfa
Increased reticulocyte count likely represents which two anemia etiologies?
Hemolysis or blood loss
Which test is most specific for hemolysis?
Haptoglobin is decreased in both extra and intravascular hemolysis
List three medications which may cause aplastic anemia.
ACE inhibitors - Sulfonamides - Phenytoin
What heritage is associated with an increased chance of thalassemia?
Mediterranean descent
What medication is commonly used for CML?
Imatinib
Translocation of chromosomes 9 and 22 or 922 suggests which disorder?
CML (This describes the Philadelphia chromosome)
WBC > 100000 and hyperuricemia are most likely associated with which disorder?
CML
Does AML primarily occur in adults or children?
Adults
The complications of multiple myeloma can be described by CRABI. List these 5 complications.
C: hyperCalcemia - R: Renal failure - A: Anemia - B: lytic Bone lesions - I: Infections
What tests are used to confirm monoclonal gammaglobulinopathy?
Protein electrophoresis + immunofixation (Monoclonal spike IgG or IgA - multiple myeloma)
Monoclonal proteinuria (Bence Jones proteins) are found in which disorder?
Multiple myeloma
A 73 year old male presents with back pain and high protein with an increased albumin/globulin ratio. Which laboratory tests would be appropriate to order next?
Serum protein electrophoresis + immunofixation and urine for Bence Jones protein in patient with suspected multiple myeloma
What is the most likely lymphoma to occur in a 31 year old male with HIV who presented to the ER after suffering focal neurological deficit?
Non-Hodgkin Lymphoma
Which is the only lymphoma that is bimodal in age of onset, painless lymphadenopathy and has Reed-Sternberg cells on pathology?
Hodgkin Lymphoma
An 82 year old male has recurrent infections, lymphadenopathy and lymphocytosis. What is the most likely type of leukemia the patient suffers from?
Chronic lymphocytic leukemia (CLL)
A patient has a leukemia with high uric acid levels and a WBC of 109,000. What chromosome abnormality would you expect to find upon further testing?
Philadelphia chromosome in patient with chronic myeloid leukemia (CML)
An acute leukemia causing lymphadenopathy, bone pain, bleeding and fever in a child is most likely which type of leukemia?
Acute Lymphoblastic Leukemia (ALL)
Which leukemia is more common in adults and on laboratory review has Auer rods with increased myeloblasts?
Acute Myeloid Leukemia (AML)
What organism associated with ground meat is often responsible for hemolytic uremic syndrome?
E. Coli 0157
What organ systems can be affected in thrombotic thrombocytopenia purpura (TTP)?
Renal, neuro and cardiac
An adult patient presents with acute thrombocytopenia, fever, multi-organ thrombosis, and hemolytic anemia. What is the best treatment for this patient?
Plasmapheresis for thrombotic thrombocytopenia purpura (TTP)
Is the onset of idiopathic thrombocytopenia purpura (ITP) usually faster or slower than TTP?
ITP has a slow onset whereas TTP is usually an acute onset
Chronic thrombocytopenia with an otherwise negative work up is most likely what disorder?
Idiopathic thrombocytopenia purpura (ITP)
What conditions other than trauma can lead to disseminated intravascular coagulation (DIC)?
Sepsis and malignancy
A patient presents 2 months after initiating warfarin with necrotic skin lesions. What underlying hypercoagulable state is most likely?
Protein C deficiency
What is the most common hypercoagulable state?
Factor V Leiden Mutation
A patient with SLE is admitted with a with new onset DVT. What antibodies should be ordered to aid in the diagnosis of the patients hypercoagulable state?
Anti-phospholipid antibodies
A 76 year old female develops a post op DVT after an ORIF if her hip. She has a platelet count of 72,000. What medication is the likely cause of her condition?
Heparin or low molecular weight heparin in patient with Heparin Induced Thrombocytopenia (HIT)
What is the difference between Von Willebrand disease and hemophilia in regards to platelet function?
Platelet function is normal in hemophilia and impaired in Von Willebrand disease
In addition to Factor VIII replacement, what medication may be used in the treatment of Von Willebrand disease?
DDAVP (desmopressin) = increased release of vWF
A 25 year old white female had prolonged bleeding after a dental procedure, bruises easily and has heavy periods. What is the most likely bleeding disorder?
Von Willebrand Disease
What anemia should be suspected in a 3 year old black male with anemia and priapism?
Sickle cell anemia
A 31 year old Mediterranean female has a hemoglobin of 10.3 gm/dl, MCV of 54, TIBC and Ferritin are normal. What is the most likely etiology of her anemia?
Thalassemia minor
Measurement of which laboratory value is the best indicator of whether or not a sickle cell crisis is resolving?
Reticulocyte count - Decreasing reticulocyte counts are the best indicator that a crisis is resolving
What medications related to travel can be an oxidative trigger for a patient with G6PD?
Anti-malarial medications
What is the most common malignancy associated with autoimmune hemolytic anemia?
CLL
A 42 year-old black female with SLE has an increased bilirubin and a mild anemia. Which test is most definitive to evaluate this patient’s anemia?
Direct coombs in patient with autoimmune anemia
Name 2 things which can cause aplastic anemia other than medications.
Chemotherapy and radiation
A patient who recently started lisinopril has a hemoglobin 7.4, WBC 1.1, platelets 55. What is the most likely disorder?
Aplastic Anemia
A dialysis patient has been on darbepoetin alfa for 3 months. His most recent hemoglobin is 11.3 gm/dl. Should he continue to use this medication?
No. erythropoetin analogs must be stopped when the hemoglobin is greater than 11 gm/dL
A 36 year old black male s/p gastric bypass surgery has peripheral neuropathy. Oral B12 has not improved his symptoms. What is the next step?
Sublingual or IM B12
What antibody test would confirm a diagnosis of pernicious anemia?
Intrinsic factor and parietal cell antibodies
A 74 year old white male with a history of chronic ETOH abuse has an MCV of 102. Physical exam finds no neurological deficit. What is the most likely diagnosis?
Folate deficiency
A 23 year old white female has weakness, fatigue and has developed a habit of chewing ice. What are the expected findings in regard to TIBC and Ferritin?
Increased TIBC and decreased Ferritin in patient with iron deficiency anemia
What organism is likely responsible for Renal failure microangiopathic hemolytic anemia and thrombocytopenia?
E. Coli 0157
