Hematology - Comprehensive Review Smarty PANCE questions

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

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

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

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

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

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

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 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)

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)

Acute chest syndrome occurs during the acute phase of which anemia?

Hemolytic sickle cell crisis

An acute leukemia causing lymphadenopathy, bone pain, bleeding and fever in a child is most likely which type of leukemia?

Acute Lymphoblastic Leukemia (ALL)

. An adult patient presents with acute thrombocytopenia, fever, multiorgan thrombosis, and hemolytic anemia. What is the best treatment for this patient?

Plasmapheresis for thrombotic thrombocytopenia purpura (TTP)

Anemia due to renal failure may be confirmed by which test?

Decreased Serum erythropoietin levels

Anemia of renal failure is defined by decreased levels of which hormone?

Erythropoietin

Auer rods?

Acute myeloblastic leukemia (AML)

Autoimmune bleeding disorder in which patients develop antibodies against their own platelets?

Idiopathic Thrombocytopenia Purpura (ITP)

Basophilic stippling of RBCs?

Lead poisoning (May also occur in thalassemia and chronic EtOH abuse) Lead poisoning (May also occur in thalassemia and chronic EtOH abuse)

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

Christmas disease is another name for deficiency of which clotting factor?

Decreased Factor IX (AKA Hemophilia B)

Chronic thrombocytopenia with an otherwise negative work up is most likely what disorder?

Idiopathic thrombocytopenia purpura (ITP)

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 C: hyperCalcemia - R: Renal failure - A: Anemia - B: lytic Bone lesions - I: Infections

Decreased Ferritin and Increased TIBC?

Iron deficiency anemia

Deficient factor in hemophilia A?

Factor VIII

Deficient factor in hemophilia B?

Factor IX

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

Does AML primarily occur in adults or children?

adults

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

Heavy menstrual cycle gum bleeding with teeth brushing?

Von Willebrand deficiency

How is factor V Leiden disorder confirmed?

Activated protein C resistance assay - Genetic testing definitive

How is hemophilia inherited?

X-linked recessive

How would you expect TIBC and ferritin to present in anemia of chronic disease?

Decreased TIBC - Normal/increased Ferritin

In addition to Factor VIII replacement, what medication may be used in the treatment of Von Willebrand disease?

DDAVP (desmopressin) = increased release of vWF

Increased MCV with smooth tongue, loss of proprioception and vibratory sense is likely which type of anemia?

B12 deficiency

Increased serum protein with a decreased albumin/globulin ratio?

Multiple myeloma

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

List 5 common causes of folate deficiency.

EtOH abuse - Advancing age - Gastric bypass - Pregnancy - Methotrexate

List the four direct thrombin inhibitors which may be used in HIT.

Lepirudin - Bivalirudin - Argatroban - Fondaparinux

List the four lab findings which support a diagnosis of DIC.

Decreased Fibrinogen - Thrombocytopenia - Increased D-Dimer - Increased PT and PTT

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 three medications which may cause aplastic anemia.

ACE inhibitors - Sulfonamides - Phenytoin

List three medications which may trigger autoimmune anemia.

Cephalosporins - Penicillins - NSAIDs

List three medications which may trigger hemolysis in a patient with G6PD deficiency.

Antimalarials - Sulfa drugs - Aspirin

List three ways to describe the genetic abnormality in CML?

922 - BCR-ABL gene - Philadelphia chromosome

List two conditions which predispose a patient to antiphospholipid syndrome.

Systemic Lupus Erythematosus - Women with recurrent abortions

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

Monoclonal proteinuria (Bence Jones proteins) are found in which disorder?

Multiple myeloma

Name 2 things which can cause aplastic anemia other than medications.

Chemotherapy and radiation

Painless lymphadenopathy indicative of which malignant disorder?

Hodgkin lymphoma

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)

A patient presents 2 months after initiating warfarin with necrotic skin lesions. What underlying hypercoagulable state is most likely?

Protein C deficiency

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 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

Philadelphia (Ph) chromosome?

Chronic myeloid leukemia (CML)

(philadelphia "cream" cheese)

Pica is a specific symptom of which type of anemia?

Iron deficiency anemia

Priapism may occur in which acute anemia?

Sickle cell crisis

Reed Sternberg cells?

Hodgkin's lymphoma

Slowly decreasing MCV in an elderly patient?

Occult GI blood loss

Translocation of chromosomes 9 and 22 or 922 suggests which disorder?

CML (This describes the Philadelphia chromosome)

TTP symptom Mnemonic: FAT-RN.

What dose FAT-RN stand for?

Fever - Anemia - Thrombocytopenia - Renal failure - Neurologic involvement

Type of anemia in which serum iron is decreased and TIBC is increased?

Iron deficiency anemia

WBC > 100000 and hyperuricemia are most likely associated with which disorder?

CML

. What anemia should be suspected in a 3 year old black male with anemia and priapism?

Sickle cell anemia

What antibody test would confirm a diagnosis of pernicious anemia?

Intrinsic factor and parietal cell antibodies

What are some common oxidative events which may trigger hemolysis in G6PD deficiency?

Antimalarials - Sulfa drugs - Aspirin - Febrile Illness - Acidosis - Fava beans

What are the organ systems which may be involved in Thrombotic thrombocytopenia purpura (TTP)?

Renal - Neuro - Cardiac

What are the two medications available for treatment of anemia due to renal failure?

epoetin alfa and darbepoetin alfa

What assay is definitive for Von Willebrand disease?

Ristocetin cofactor assay

What conditions other than trauma can lead to disseminated intravascular coagulation (DIC)?

Sepsis and malignancy

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 disorder is usually diagnosed early in childhood and has hemarthrosis bruising and bleeding?

Hemophilia

What disorder should be considered for a patient on warfarin who develops diffuse necrotic skin lesions?

Protein C deficiency

What do an increased LDH, decreased Haptoglobin and an increased indirect Bilirubin represent?

Hemolysis

What does a > 50% reduction in platelet count within 7-10 days of exposure to heparin define?

Heparin Induced Thrombocytopenia (HIT) syndrome

What does the presence of Heinz bodies indicate?

G6PD deficiency (May also be found in thalassemia)

What factor is produced in the stomach and required for Vitamin B12 absorption?

Intrinsic factor

What factors are affected in Von Willebrand disease?

Von Willebrand factor and factor VIII are both decreased

What gene mutation is associated with polycythemia vera?

JAK2 mutation

What hematological malignancy has a bimodal incidence by age of 15-45 years old or >60 years old?

Hodgkin lymphoma

What heritage is associated with an increased chance of thalassemia?

Mediterranean descent

What is first line treatment for Idiopathic thrombocytopenia purpura?

Steroids

What is the acute treatment of Von Willebrand disease?

Acute/initial treatment: DDAVP

What is the best test to determine whether a patient as a hemolytic anemia?

Reticulocyte count and haptoglobin

What is the chronic treatment of Von Willebrand disease?

Chronic treatment: Factor VIII replacement

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

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 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 Dx? Teenage African American with severe bone and joint pain severe anemia.

Sickle cell disease

What is the Dx? Very low MCV, normal/high RBC, normal RDW and high/normal serum iron normal TIBC.

Thalassemia

What is the etiology of pernicious anemia?

Antibodies to gastric parietal cells and intrinsic factor

What is the most common autoimmune disorder associated with hemolytic anemia?

Systemic lupus erythematosus (SLE)

What is the most common genetic hypercoagulable state?

Factor V Leiden

What is the most common hypercoagulable state?

Factor V Leiden Mutation

What is the most common malignancy associated with autoimmune hemolytic anemia?

CLL

What is the most common type of kids leukemia?

Acute lymphocytic leukemia (ALL)

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

What is the result of homozygous alpha thalassemia (Alpha major) genetics?

Fetal death