Heme 1

Question 1

Mucocutaneous Bleeding, normal platelet Count.

Next step?

Answer 1

Platelet Function Analyzer. (SSRIs and Ginko can affect plt dysfunction)

Question 2

LE swelling, SOB, Elevated D-dimer, Pulmonary Embolism severity index score (PESI) 59.
Rx?

Answer 2

Apixaban and Rivaroxaban (Eliquis and Xarelto) - Requires NO Bridging

Index score less than 65 Managed Outpatient (age, sex, current cancer, heart failure, lung disease, pulse, systolic, 02 sat, RR, temp, stupor/lethargy) can be managed outpatient.

Question 3

Rx for Myelodysplastic Syndromes that require frequent Transfusions?

Answer 3

Lenalidomide (used in 5q deletions)

(Helps with Transfusion Dependence and Progression to AML)

Question 4

Rx of choice with in pts with PE and Active Cancer?

Answer 4

LMWH (Enoxaparin)

Question 5

Management of patient with MGUS and M spike of less than 1.5, normal SPEP and UPEP?

Answer 5

Repeat labs in 6 months

Risk of progression to MM and other Lymphoproliferative disorders is low.

Question 6

Rx for patients with Acquired Hypogammaglobulinemia from MM with recurrent sinusitis?

Answer 6

IVIG to provide Passive immunity.

Question 7

Pt presents with Scleral icterus, Fever, Tea Colored urine, LOW Hemglobin and Elevated bilirubin 10 days after being transfused.

Dx? What test confirms this?

Answer 7

Delayed Hemolytic Transfusion Reaction

Confirmed by positive Direct Antiglobulin (Coombs test) DAT

Antibodies bind to antigen and agglutin

Question 8

Pt with Sick cell Anemia Presents with SOB, fatigue, Hemoglobin of 4 and retic count of 0.1
Dx?

Answer 8

Parovirus B19 - Aplastic Crisis

(affects Erythrocyte Precursors in bone marrow)

Question 9

Rx for Essential Thrombocytosis?

What test should be done?

Next step if Negative?

Rx for P Vera?

Answer 9

Aspirin and Hydroxyurea.

Check for Jak2 Mutation

IF negative then Bone Marrow Biopsy

Phlebotomy (Get Hematocrit Down), Aspirin and Hydroxyurea- Prevent thrombosis

Question 10

Black ppl WBC 1.8 and NO Symptoms

Dx?

Answer 10

Benign Ethnic Neutropenia

Question 11

Edema, Dizziness, Indentations on side of tongue, JVD, Orthostatic Hypotension, Decrease breath sounds, S3 gallop, Elevated Cr, Albuminuria, IgG lambda spike, THICKENED left ventricular wall, Diastolic dysfunction

Dx?
Rx?

Answer 11

Amyloidosis (infiltrative Disorder)

Abdominal fat pad biopsy

Question 12

Hepatosplenomegaly, Night sweats, Anemia, Hyperviscosity, IgM spike

Dx?
What confirms diagnosis?
Rx?

Answer 12

Waldenstrom Macroglobulinemia

• 1st M spike on Electrophoresis
• Then Immunofixation to identify which abnormal protein it is (IgM)
• Bone marrow biopsy

Plasmapharesis

Question 13

Rx for DVT with acute limb ischemia?

Answer 13

Alteplase

Question 14

1. Serum Monoclonal M protein < 3 and Bone marrow clonal plasma cells < 10%
   No symptoms?
2. Pt presents with serum M protein of >3 g/dl or 500mg/24 hr of urinary Monoclonal Free light chains or Bone Marrow Plasma Clonal cells > 10% or greater and NO other symptoms.
   Dx?
3. Plasma cells > 10% with Protein > 3 + symptoms of BONE PAIN?
   Dx?

Answer 14

1. MGUS
2. Smoldering
3. MM

MGUS <10% Plasma Cells) —-> Smoldering (> 10% Plasma Cells) —> MM (>10% Plasma Cells + Symptoms)

Question 15

1. INR 4.5 - 10 and No bleeding
2. > 10 and No Bleeding
3. > 10 and Bleeding

Answer 15

1. Omit Dose
2. Vitamin K
3. Vit K 10mg IV + Four Factor PCC

Question 16

Rx for secondary Iron Overload from frequent transfusions Dx?

Answer 16

Deferasirox (oral)/Deferoxamine (IV)

Question 17

What is the Rx of Anemia of ckd pts?

Answer 17

Epo stimulating agents (darbepoetin) to avoid increased Cardiovascular Events caused by Frequent Transfusions.

Question 18

Pt with Minor Bleeding while on Pradaxa? Next step

Answer 18

Observation (wait 24 hrs before finding cause of bleeding)

Question 19

Test to r/o probable PE and Avoid unnecessary D- Dimer checking?

Answer 19

Wells Criteria and PERC

Age< 50years
HR < 100
O2 sat 95%,
No hemoptysis
No Leg Swelling
No Estrogen use
No Previous DVT/PE
No Surgery or Trauma
No Recent hospitalization in last 4 weeks

Symptoms, Vitals, Medical Hx

Question 20

Chronic Hemolytic Anemia (smear shows no Schistocytes, Spherocytes, immature appearing leukocytes, Agglutinate Erythrocytes), Red Urine (Hemoglobinuria), Venous Thrombosis and Pancytopenia.

Dx?
Test?
Rx?
Treatment if Young?

Answer 20

1. Paroxysmal Nocturnal Hemoglobinuria (PNH)

Flow Cytometry looking for cell surface markers CD55, CD59.

Chemo: Eculizumab
Allogenic HSCT if < 50 years
Rx iron, folate as indicated.

Question 21

1. Management of sickle cell patients who have Uncomplicated Pregnancies?
2. What is a known complication of Pregnancy in patient with Sickle Cell Dz?

Answer 21

Monitor

Pulmonary HTN, Evaluated with Echo

Question 22

1. Low serum ferritin, low RBC count, High RDW, normal hgb electrophoresis ?
2. Target cells, Normal serum ferritin, normal or High RBC count, Normal RDW, Normal hemoglobin electrophoresis?
3. Target cells, Normal serum ferritin, Normal or High RBC count, Normal RDW, Elevated Hgb A2 and Fetal hemoglobin (Hgb F) on electrophoresis?

Answer 22

1. Iron deficiency
2. Alpha-thal
3. Beta-thal

Question 23

1. 90% Hb S and no HbA?
2. HbS 30% and HbA 50%?

Answer 23

1. Sickle cell Dz
2. Sickle Cell Trait (no painful cries)

Question 24

Pt hospitalized for PNA, treated with Ceftriaxone, Hgb 6.5g/dl, Elevated LDH, Spheroctyes on smear and Direct Antiglobulin test POSITIVE or IgG ?

Answer 24

Drug Induced Hemolysis

Question 25

1. Hemolytic anemia caused by IgG, IgG positive, C3 positive or negative on DAT, spheroctyes present on smear, Aggulinates in > 37degree ? Dx? Rx? 2. Hemolytic anemia (caused by IgM), IgG negative, C3 POSITIVE on DAT, RBC agglutination on smear when Cold? Rx? Dx?

Answer 25

1. Warm autoimmune Hemolytic Anemia Steroids IVIG Rituximab OR Splenectomy 2. Cold Agglutinin Disease (Mycoplasma, EBV) Avoid Cold Exposure Warm saline Steroids This mainly occurs Extravascularly (IVIG, Splenectomy Not helpful)

Question 26

1. Respiratory distress within 6 hrs of transfusion, positive fluid balance, elevated BNP and CXR showing pulmonary edema? 2. Respiratory distress, dyspnea, hypoxia, no JVD within 6 hrs of transfusion and CXR showing B/L Pulmonary Infiltrates? 3. Occurs when donor plasma constituents react with recipients IgE on mast cells, causing rash, hives, wheezing, mucosal edema? 4. Rare but fatal complication in which donor lymphocytes...... immunocompromised or HLA similar recipient and cause reactions that affect bone marrow, liver, skinned GI tract?

Answer 26

1. Transfusion Associated Circulatory Overload. (TACO) 2. Transfusion Related Acute Lung Injury (TRALI) 3. An Allergic Rransfusion Reaction 4. Transfusion-associated Graft-Versus-Host- Disease

Question 27

Reversal agent for Dabigatran? Reversal Agent for Warfarin?

Answer 27

Idarucizumab Prothrombin Complex. (Kcentra)

Question 28

Alcohol dependence, low Hgb, MCV > 110, Hypersegmented Neutrophils on smear. Dx?

Answer 28

Folate deficiency! B12 Deficiency (Effectively Stored) not seen with alcohol Dependence

Question 29

Elevated CA, Renal failure, Anemia and Bone disease. Dx? Test? Rx? How do you Managed Lytic bone lesions?

Answer 29

MM SPEP Immunofixation Skeletal Survey Chemotherapy : Bortezomib, Lenalidomide, Dexamethasone, IV Zoledronic Acid.