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Hematology > MKSAP Heme > Flashcards

MKSAP Heme Flashcards

1
Q

What does Idarucizumab used for? Andexanet?

A

Reverse dabigatran

Reverse DOACs

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2
Q

How do you treat aplastic anemia?

A

Stem cell transplant

Antithymocyte globulin, cyclosporine, prednisone

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3
Q

What are some acquired causes of pure red cell aplasia? Name 5.

A

Thymoma, autoimmune disease, malignancy (lymphoma, leukemia, solid tumors) drugs (phenytoin, INH), pregnancy, anti-EPO antibodies in patients receiving EPO

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4
Q

What are two treatment options for patients with MDS that are high risk?

A

Azacytidine and Decitabine

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5
Q

What is the treatment for low risk MDS patients with -5q cytogenic abnormality?

A

Lenalidomide

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6
Q

What are some causes of secondary polycythemia? Name 8.

A

Hypoxemia mediated: COPD, OSA, intrapulmonary shunting, congenital heart disease, elevated altitude
Ectopic or excess EPO: RCC, HCC, renal artery stenosis, uterine fibroids
Testosterone

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7
Q

What is the management for polycythemia vera?

A

ASA for all patients
Hydroxyurea for those at high risk of thombosis (age >60 or hx of thrombosis)
Ruxolitinib (tyrosine kinase inhibitor) for those intolerant of the above or with refractory PV

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8
Q

What is the management for essential thrombocythemia?

A

Hydroxyurea for patients age >60, platelets >1 million, and/or hx of thrombosis
ASA for all other patients
Anagrelide and interferon-alpha are alternatives for those who do not respond to hydroxyurea

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9
Q

What is the treatment for primary myelofibrosis?

A

Allogenic hematopoietic stem cell transplant
Ruxolitiniib for those not candidates for transplant
Hydroxyurea for low risk PMF

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10
Q

What are 5 causes of eosinophilia? (CHINA mnemonic)

A 
Collagen vascular disease
Helminthic infection
Idiopathic hypereosinophilic syndrome
Neoplasia (lymphoma most common; MPN)
Allergy, atopy, asthma; also drug induced (carbamazepine, sulfonamides)
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11
Q

What type of amyloid protein is seen in the following amyloidosis types? AL amyloidosis, hereditary, AA amyloidosis, age-related, dialysis-related?

A 
AL: monoclonal free kappa or lambda light chains
Hereditary: mutated transthyretin (TTR)
AA: serum amyloid A protein
Age-related: wild type TTR
Dialysis-related: beta 2 microglobulin
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12
Q

What diseases are AL amyloidosis and AA amyloidosis associated with?

A

AL: plasma cell dyscrasias (MGUS, MM), waldenstrom macroglobulinemia
AA: RA, IBD, familial mediterranean fever, chronic infection

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13
Q

What is the treatment for AL amyloidosis?

A

Autologous HSCT
Melphalan or Bortezomib based chemotherapy if ineligible for transplant
Can also use similar treatment regimens as multiple myeloma

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14
Q

What are 3 criteria for Waldenstrom macroglobulinemia?

A

> 10% clonal lymphoplasmacytic cells in bone marrow
3 g/dL of monoclonal protein (IgM)
Symptoms from disease

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15
Q

What are possible treatment options for waldenstrom macroglobulinemia? Name 4.

A

Plasmapheresis to remove excess IgM
Glucocorticoids
Ibrutinib
Rituximab, either single agent or in combo with other agent

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16
Q

What are the differences between type I, II, and III cryoglobulinemia?

A

Type 1 involves a monoclonal immunoglobulin, usually IgM; associated w/ plasma cell dyscrasias and typically is asymptomatic; may present w/ hyperviscosity or thrombosos; livedo reticularis or purpura is on exam
Types 2 & 3 are mixed - polyclonal IgG and monoclonal or polyclonal IgM; has low C4; associated w/ hepatitis C, HIV, connective disorders, or lymphoproliferative disorders; presents w/ fevers, arthralgias, GN, HTN, dyspnea, palpable purpura, LCV

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17
Q

Patients w/ PNH develop episodic hemolysis, marrow aplasia, and thrombosis. What is the management for PNH?

A

Eculizumab for symptomatic patients; is associated w/ Neisseria infections so need meningococcal vaccine
Prophylactic anti-coagulation, iron, folic acid

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18
Q

In which disease are clonal CD57-positive T cells consistent w/ large granular lymphocytosis found? What is the treatment for when this is found?

A

Secondary pure red cell aplasia

Methotrexate or cyclosporine

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19
Q

What is the treatment for primary hypereosinophilic syndrome?

A

Steroids

Imatinib if patients has activating mutation of platelet derived growth factor receptor (PDGFR)

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20
Q

What are some characteristics of thalassemia that help differentiate it from IDA?

A

Uniform cells in shape and size, red cell distribution width is normal;
Elevated LDH, elevated unconjugated bilirubin, decreased haptoglobin

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21
Q

What occurs with the gene mutations in alpha thalassemia (1 through 4)?

A

1 mutation - silent carrier and healthy
2 gene mutation - alpha-thal trait; Hgb ~10 with microcytosis
3 gene mutation - tetramer of beta globin called hemoglobin H; Hgb7-8
4 gene mutation - intrauterine demise

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22
Q

What are two new treatments that can be used to prevent painful crises in SCD?

A

L-glutamine

Crizanlizumab

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23
Q

What is the pre-op Hgb goal for sickle cell disease patients?

A

10

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24
Q

What are the indications for exchange transfusion in SCD? Name 3. What is the goal Hemoglobin S?

A
  1. Acute stroke, complicated acute chest syndrome (O2 <85% or >2 pulmonary lobes involved), and acute retinal artery occlusion
  2. Goal Hemoglobin S <30%
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25
Q

For warm autoimmune hemolytic anemia what is the Ig class, peripheral smear findings, associated conditions (name 3 diseases and 5 drugs), complications, and treatment?

A

IgG; C3 positive or negative
Spherocytes
Conditions: autoimmune (SLE), lymphoproliferative (CLL, B cell non hodgkin lymphoma), drug induced (penicillin, methyldopa, isoniazid, NSAIDs, cephalosporins, levodopa)
Complications: venous thromboembolism
Tx: Steroids, IVIG, rituximab; splenectomy

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26
Q

For cold agglutinin disease what is the Ig class, peripheral smear findings, associated conditions (name 3), complications, and treatment?

A

IgM; C3 positive (so test for direct coombs for C3)
Erythrocyte agglutination (leading to markedly elevated MCV)
Infection (mycoplasma, EBV), lymphoproliferative (IgM MGUS, waldenstrom macroglobulinemia, other B cell non hodgkin lymphoma)
Complications: ischemia and peripheral gangrene; lymphoproliferative disorders
Cold avoidance; Rituximab, plasmapheresis, tx underlying condition

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27
Q

What are some drugs that can cause TTP? Name 4.

A

Gemcitabine, Cyclosporine, Bevacizumab, Ticlodipine, Quinine, Clopidogrel

28
Q

What are eltrombopag and romiplostim?

A

Thrombopoietin receptor agonist

29
Q

What are the differences between type 1 & type 2 HIT? How do you test for HIT?

A
  1. Type 1 is non-immune mediated and occurs within a few days of heparin exposure, and no intervention is needed
    Type 2 is immune-mediated and occurs 5-10 days after exposure
  2. Platelet factor 4 antibodies; confirm diagnosis w/ serotonin release assay or heparin-induced platelet aggregation assay
30
Q

What are alternative anti-coagulants that can be used in HIT? Name 3. How long should they be continued?

A

Argatroban, Bivalirudin, Fondaparinux, Rivaroxaban, Danaparoid
2-3 months without documented thromboembolic events, 3-6 months for patients w/ associated thrombosis

31
Q

What are some causes of prolonged PTT + normal PT?

What are some causes of prolonged PT & PTT? Name 5.

A
  1. vWD, heparin exposure, deficiency of factors VIII, IX, XI, XII
  2. Deficiency of factors V, X, II or fibrinogen; severe liver disease, DIC, vitamin K deficiency, warfarin toxicity, heparin overdose
32
Q

What is the treatment for bleeding in hemophilia A?

A

Desmopressin for mild hemophilia A

Aminocaproic acid and tranexamic acid for bleeding from dental procedures

33
Q

What are the types of vWD and treatment? How do you screen for vWD?

A

Type 1: low vWF (<30%) but normal function; treat w/ desmopressin
Type 2: abnormal vWF function; treat type 2A w/ desmopressin or vWF concentrates, treat type 2B w/ vWF concentrates
Type 3: severe deficiency of vWF, presents w/ joint and deep muscle bleeding; treat w/ vWF concentrates
Screening: prolonged platelet function analyzer closure time, normal or prolonged aPTT

34
Q

What are some conditions that can lead to acquired hemophilia, with antibodies against factor VIII? What is the management?

A
  1. Pregnancy, post-partum, malignancy, and autoimmune disease
  2. Activated prothrombin complex concentrates, or activated factor VII to overcome the inhibitor; corticosteroids and cyclophosphamide
35
Q

What is in cryoprecipitate?

A

Fibrinogen and factor VIII

36
Q

What are the indications for the following in transfusions? Leukoreduction (decreases number of leukocytes), Irradiation (prevents replication of lymphocytes and stem cells), Washing (removes proteins)?

A

Leukoreduction: reduces alloantibody production, decreases febrile non-hemolytic transfusion reactions, decreases transmission of CMV ; also use in potential transplant recipients or chronically transfused patients

Irradiation: prevents GVHD; indicated in severe immunodeficiency, or in those receiving HLA-matched platelets/transfusions from relatives

Washing: hx of severe allergic reactions, IgA deficiency, complement dependent autoimmune hemolytic anemia

37
Q

What should be tested for in patients with an anaphylactic transfusion reaction?

A

IgA or haptoglobin deficiency

38
Q

How long after pregnancy should patients w/ hx of idiopathic VTE, VTE associated w/ pregnancy or OCPs, or hereditary thrombophilia be treated w/ anti-coagulation?

A

6 weeks

39
Q

What is the most common inherited thrombophilila and what is the pathophysiology?

A

Factor V Leiden
Factor V combines w/ factor X to produce thrombin, which leads to clot formation; this is regulated by protein C to stop the process of ongoing clot formation
Factor V Leiden is resistant to cleavage by protein C -> predisposition of thrombus formation

40
Q

What pro-thrombotic condition should be considered if titration to a therapeutic range for heparin is difficult? What are some acquired causes of this condition?

A
  1. Anti-thrombin III deficiency

2. Liver disease, nephrotic syndrome, protein-losing enteropathy, heparin therapy, acute thrombosis

41
Q

What are some acquired causes of protein C and protein S deficiencies?

A

Protein C: acute thrombosis, warfarin therapy, liver disease, protein-losing enteropathy
Protein S: acute thrombosis, warfarin therapy, liver disease, inflammatory states, estrogens, protein-losing enteropathy

42
Q

What are the 3 antibodies tested in antiphospholipid syndrome?

A

Lupus anti-coagulant, anticardiolipin antibodies, anti-beta2-GP antibodies

43
Q

What are the indications for treatment of lower extremity superficial venous thrombosis? Name 3.

A

5 cm in length, close to deep venous system, or other thrombophilic risk factors (cancer, previous DVT)
Treat w/ fondaparinux or alternative

44
Q

What is the MOA of unfractionated heparin? What is the reversal agent used, dose of the agent, and its side effects?

A
  1. Binds antithrombin -> inactivation of thrombin and factor Xa
  2. Protamine sulfate , 1 mg per 100 units of heparin
  3. Side effects: allergic reactions, hypotension, bradycardia, respiratory toxicity
45
Q

What is the treatment for chronic parvovirus infection w/ anemia?

A

IVIG

46
Q

What is the most sensitive test for PNH diagnosis?

A

FLAER (fluorescein-labed proaerolysin) - lack of this confirms diagnosis (since proaerolysin binds to GPI anchor, and PNH cells lack this)

47
Q

What does the eosin-5-maleimide binding test +/- acidified glycerol lysis time or cryohemolysis test used for?

A

Hereditary spherocytosis diagnosis

48
Q

What is seen on hemoglobin electrophoresis in alpha thal trait and beta thal trait?

A

Alpha thal trait: normal electrophoresis

Beta thal trait: increased hemoglobin A2 and hemoglobin F

49
Q

What are 3 lab findings (not on CBC) found in primary myelofibrosis?

A

Elevated LDH, uric acid, and alkaline phosphatase

50
Q

In what thrombophilic disorder are patients more likely to develop warfarin-induced skin necrosis?

A

Protein C deficiency
When warfarin is initiated, there is an initial reduction in protein C activity of 50% leading to a transient hypercoagulable state

51
Q

What is the management of transient aplastic crisis in hereditary spherocytosis?

A

Observation

Can occur with acute infection

52
Q

Which factor is normal or elevated in liver disease, but low in DIC?

A

Factor VIII - it is produced in extrahepatic endothelial cells, and a factor that is produced in the liver is needed for clearance

53
Q

What are the 3 mutations found in essential thrombocythemia?

A

JAK2 (50% of patients)

Calreticulin, MPL genes

54
Q

How does hemoglobin S beta thalassemia present?

A

Mild hemolytic anemia, microcytosis, hemoglobin A between 5-30%, hemoglobin S levels ~60%

55
Q

What is Eculizumab used for?

A

PNH and atypical HUS

56
Q

What is the initial workup for a patient w/ abdominal thrombosis, splenomegaly, and portal HTN?

A

Evaluate for JAK2 mutation, even in absence of thrombocytosis or erythrocytosis
50% of Budd-Chiari cases, 25% of portal vein thrombosis cases are due to myeloproliferative neoplasm

57
Q

In which patients should 4f-PCC be avoided?

A

Those w/ history of HIT b/c it contains residual heparin

58
Q

How does osler-weber-rendu syndrome or hereditary hemorrhagic telangiectasia present?

A

Frequent epistaxis, multiple mucocutaneous telangiectasias (including in GI tract leading to bleeding); AVMs in liver (biliary disease, portal HTN, high output HF), brain (HA, seizures, hemorrhage, stroke), or lungs (cyanosis, dyspnea, paradoxical embolism, cerebral abscess)

59
Q

What lab is checked to confirm someone is anti-coagulated on dabigatran?

A

Thrombin time

60
Q

What is the management for priapism lasting <2 hours? >2 hours? >12 hours?

A
  1. Increased fluids, oral analgesics, attempted urination
  2. IV pain medication, hydration, blood aspiration from corpus cavernosum w/ saline irrigation, injection of phenylephrine or epinephrine to restore venous outflow
  3. Glans-spongiosum shunt
61
Q

What can reduce subsequent stroke risk for a patient with sickle cell disease who has had a stroke?

A

Monthly simple (or exchange) transfusions

62
Q

How does bone marrow aspiration w/ prussian blue help differentiate between iron deficiency and anemia of chronic disease?

A

In ACD will have no iron staining in the red cell precursors but will have abundant staining in the macrophages (due to iron trapping)
In IDA there will be no iron staining in either macrophages or red cell precursors

63
Q

Approximately how much hemoglobin S is found in sickle cell disease? S-beta-thalassemia? Sickle cell trait?

A
  1. > 90%
  2. ~60%
  3. ~30%
64
Q

What is the recommended dose of FFP to correct coagulopathy?

A

15 ml/kg

250 mL in each unit

65
Q

What kind of monitoring is required when iron chelation (deforxamine and deferasirox) are used?

A

Kidney, liver, CBC, eye

Hematology (1 decks)

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