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Hematology Board Review

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Hematology Board Review Alice Ma, MD UNC Hematology-Oncology June 9, 2006 Case 5 The patient has an acquired coagulation disorder that is associated with bleeding. – PowerPoint PPT presentation

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Title: Hematology Board Review


1
Hematology Board Review
  • Alice Ma, MD
  • UNC
  • Hematology-Oncology
  • June 9, 2006

2
Case 1 (Item 22 from MKSAP)
  • 68 y.o. woman from Minnesota seen in ER for
    fatigue, DOE, and episodic dark urine. Dark
    urine noted following extended periods of time in
    the cold. No medications
  • PEX. T nl, HR 90, BP 110/75
  • Hgb 7.1, retic count 18. Normal indices, normal
    remainder of CBC
  • Lab notes sample appears agglutinated, and
    clumping of RBCs on smear.

3
Case 1
  • 68 y.o. woman from Minnesota seen in ER for
    fatigue, DOE, and episodic dark urine. Dark
    urine noted following extended periods of time in
    the cold. No medications
  • PEX. T nl, HR 90, BP 110/75
  • Hgb 7.1, retic count 18. Normal indices, normal
    remainder of CBC
  • Lab notes sample appears agglutinated, and
    clumping of RBCs on smear.

4
Case 1
  • Which of the following is the most important
    study for establishing the diagnosis in this
    patient?
  • A. Osmotic fragility
  • B. Cold Agglutinin screen
  • C. Serum complement determination
  • D. Sucrose hemolysis test
  • E. G6PD determination

5
Case 1
  • A - No, since this is for hereditary
    spherocytosis.
  • Buzzwords for HS
  • Recurrent jaundice
  • Early pigment gallstones, cholecystectomy
  • Autosomal dominant, so FHx
  • Splenomegaly
  • Increased osmotic fragility

6
Case 1
  • B - Cold agglutinin Screen. Yes
  • Buzzwords for cold agglutinin disease
  • Recurrent hemolysis in the cold
  • RBC clumping on smear
  • DAT () for C3/complement, (-) for IgG
  • May follow infection with mycoplasma or mono
  • IgM mediated
  • No benefit from steroids or splenectomy
  • Keep warm

7
Case 1
  • C. Sucrose Hemolysis test. No. This is for PNH
  • Buzzwords for PNH
  • Dark/red urine in early AM
  • May have clots and/or pancytopenia
  • May follow chemo/aplastic anemia
  • Tests
  • sucrose hemolysis test
  • Acidified serum hemolysis test (Hams test)
  • Flow cytometry for CD55/CD59
  • Urine hemosiderin positive

8
Case 1
  • E. G6PD determination
  • Buzzwords for G6PD deficiency
  • African-American or Mediterranean
  • X-linked
  • Hemolysis follows infection or drugs
  • Susceptible to aplastic crisis after parvo
  • Drugs - sulfa/dapsone/antimalarials
  • Heinz bodies (need special stain)
  • Bite cells

Blister cells
9
More Hemolytic Anemia Buzzwords
  • Warm AIHA
  • Spherocytes
  • Positive DAT, IgG positive, C3 positive
  • Associated with CLL, NHL, SLE
  • Treat with steroids first, splenectomy second.
  • Drug-related AIHA
  • Spherocytes.
  • Positive DAT, usually for C3 only.

10
Case 2 (Item 55 from MKSAP)
  • 22 y.o. man brought from work to ER for abdominal
    pain and fever.
  • Two recent episodes of red urine
  • Previously treated for syphilis
  • Works as a butchers assistant, unloading
    refrigerated meat trucks
  • PEx - chronically ill. Nl Temp and BP

11
Case 2
  • 22 y.o. man brought from work to ER for abdominal
    pain and fever.
  • Two recent episodes of red urine
  • Previously treated for syphilis
  • Works as a butchers assistant, unloading
    refrigerated meat trucks
  • PEx - chronically ill. Nl Temp and BP

12
Case 2
  • Laboratory studies
  • Hgb 4.0
  • WBC nl. Nl diff except for NRBCs
  • Plts nl
  • Rapid Plasma reagin test - positive
  • U/A. Strongly positive for hemoglobin. No
    intact erythrocytes

13
Case 2
  • Which of the following is the most appropriate
    diagnostic study for this patients hemolytic
    anemia?
  • A. Donath-Landsteiner test
  • B. Sickle Cell Preparation
  • C. Urine Hemosiderin preparation
  • D. Heinz body preparation

14
Case 2
  • B. Sickle Cell preparation - no
  • U/A shouldnt be red, may have microscopic
    hematuria, not hemoglobinuria
  • No association with syphilis
  • Peripheral smear should show sickled cells,
    especially if pt is quite ill now

15
Case 2
  • C - urine hemosiderin determination. No, since
    we know that there is hemoglobin in the urine.
    Urine hemosiderin is useful if there is the
    suspicion for chronic intravascular hemolysis, as
    in PNH, valve hemolysis, AAA/aortic dissection
    hemolysis.

16
Case 2
  • D. Heinz body preparation - no
  • This is useful if there is suspicion for
    oxidative stress, usually from drugs.
  • bite cell hemolytic anemia
  • Usually G6PD deficiency, but if oxidative stress
    is bad enough, anyone can get Heinz body
    hemolytic anemia.
  • African americans with G6PD deficiency can have
    falsely nl G6PD levels immediately after hemolysis

17
Case 2
  • A - Donath Landsteiner test - correct
  • Paroxymal Cold Hemoglobinuria (PCH)
  • Episodic cold-induced intravascular hemolysis
  • DAT positive only for Complement
  • Seen in pediatrics, also classically with
    syphilis, now most are idiopathic
  • IgG antibody binds only in the cold, but fixes
    complement. No spherocytes
  • Special test for DL antibody detection.

18
Case 3 (Item 65 from MKSAP)
  • 22 y.o. man seen in the ER for red urine and
    fatigue. 3 days ago, started on TMP-SMZ for UTI.
  • Hbg 6.5 retic 18
  • Blood smear - polychromatophilia, blister cells
  • G6PD - low normal

19
Case 3
  • In order to confirm the diagnosis, which of the
    following should be done?
  • A. repeat G6PD determination in 1 month
  • B. perform osmotic fragility test
  • C. perform sucrose hemolysis test
  • D. perform bone marrow aspirate

20
Case 3
  • Two normal forms of enzyme. Most prevalent type
    is B. 20 of healthy Africans have type A.
  • Deficiency is X-linked.
  • In Africans, mutant protein is A-, which is
    unstable and loses activity as the red cell ages.
  • Mediterranean variant has baseline low activity
  • Low G6PD activity results in low levels of NADPH
    and reduced glutathione, which are required to
    protect hemoglobin from oxidative damage.

21
Case 3
  • Typically, hemolysis can be triggered by drugs or
    infections.
  • Anemia is maximal 7-10 d after exposure. In
    individuals with A-, reticulocytosis begins to
    compensate for the anemia, despite continuation
    of the drug.
  • Immediately after a hemolytic episode, G6PD
    levels in the with A- may be normal, since the
    mature cells have been lysed, and only younger
    cells with normal G6PD levels, are present. Need
    to repeat in 1 month

22
Case 3 - blister cells
23
Case 4
  • A 27-year-old woman is referred to you for
    evaluation of thrombocytopenia, which was
    discovered incidentally during an evaluation for
    life insurance.
  • She is healthy, active, and taking no
    medications.
  • Findings on the history and physical examination
    are normal.
  • The complete blood count results are normal,
    except for a platelet count of 72,000/?L.
  • The blood chemistry profile is normal, urinalysis
    is normal, and the test for HIV is negative.
  • Examination of the peripheral blood smear is
    normal except for the mild thrombocytopenia.

24
Case 4
  • What is the most appropriate management for this
    patient?
  • (A) Suspect ITP. do no further diagnostic
    studies other than a repeat complete blood
    count in 1 month.
  • (B) Suspect ITP begin prednisone, 1 mg/kg/d
  • (C) Suspect ITP or possibly myelodysplasia do a
    bone marrow aspiration and biopsy.
  • (D) Suspect the possibility of lymphoma do a CT
    of the chest and abdomen for lymphadenopathy.
  • (E) Doubt the history and suspect alcoholism
    do a liver and spleen ultrasonography.

25
Case 4
  • In the absence of any gold standard tests to
    diagnose ITP, the diagnosis can only be based on
    the observation of thrombocytopenia without other
    hematologic abnormalities.
  • Also, recognize that the goal for treatment of
    ITP is only to prevent bleeding, which is not an
    issue in this woman with mild thrombocytopenia.

26
Case 4
  • Prednisone therapy
  • the initial choice for adults with ITP
  • does not induce a cure
  • may only provide a temporary remission or provide
    symptomatic relief until a spontaneous remission
    occurs.
  • Therefore, prednisone is not indicated in the
    absence of thrombocytopenia that poses no
    hemostatic risk.
  • Prednisone may be appropriate if the platelet
    count is less than 20,000 to 30,000/?L.

27
Case 4
  • When there are no other hematologic abnormalities
    on routine CBC and on peripheral blood smear,
    examination of the bone marrow does not provide
    additional important information.
  • It is unnecessary to routinely do a bone marrow
    examination in the evaluation of a patient with
    suspected ITP.
  • If pt were older or had abnormal counts other
    than plts, marrow exam may have been appropriate.

28
Case 4
  • Other etiologies for thrombocytopenia such as
    occult liver disease with hypersplenism or occult
    lymphoma may be considered, but in the absence of
    a suggestive history or physical examination,
    these possibilities warrant no further diagnostic
    evaluation.
  • Alcohol excess itself may cause thrombocytopenia
    due to marrow suppression.

29
Case 4
  • ITP therapy in adults
  • First - if plts lt 30-40, or if pt bleeding, use
    steroids /- IVIg to raise platelet count, await
    spntaneous remission.
  • If pt relapses after steroid taper, move to
    splenectomy - effective in two thirds of pts.

30
Case 4
  • If pt fails splenectomy, lower your target plt
    count to 30. Use immunosuppression at this
    point. This can include azathioprine,
    cyclophosphamide, cyclosporine,
  • Also can try danazol, protein A column,
  • If pt initially responds after splenectomy, then
    relapses after, consider accessory spleen.

31
Case 5
  • A 76-year-old man presents to the emergency
    department with numerous ecchymoses and bleeding
    from the gums.
  • He has been previously well, and he takes no
    medications.
  • There is a history of uncomplicated appendectomy
    and cholecystectomy.
  • On physical examination, he has extensive
    bruising, and there is oozing from intravenous
    puncture sites. In addition, he has a swollen,
    painful left knee.

32
Case 5
  • Laboratory studies
  • Platelet count 264,000/?L
  • Activated partial 112 sec thromboplastin time
  • Prothrombin time 11 sec
  • Plasma fibrinogen 2.4 g/L
  • A 11 mix of patient and normal plasma does not
    correct the prolonged activated partial
    thromboplastin time.
  • The patient had an activated partial
    thromboplastin time of 28 seconds at the time of
    his appendectomy 12 years ago.

33
Case 5
  • The most likely cause of this patients
    coagulopathy is
  • (A) Disseminated intravascular coagulation
  • (B) A lupus anticoagulant
  • (C) Factor XII deficiency
  • (D) Acquired prothrombin inhibitor
  • (E) Acquired factor VIII inhibitor

34
Case 5
  • The patient has an acquired coagulation disorder
    that is associated with bleeding.
  • Although disseminated intravascular coagulation
    (DIC) is associated with prolongation of the
    activated partial thromboplastin time, this
    diagnosis is unlikely given the normal platelet
    count, prothrombin time and fibrinogen level.
    Also there is no associated systemic illness that
    could provoke DIC.
  • Factor XII deficiency is ruled out by the
    presence of bleeding, because factor XII
    deficiency does not cause bleeding.

35
Case 5
  • Lupus anticoagulants are not associated with
    hemorrhage unless there is associated deficiency
    of prothrombin. This possibility is excluded by
    the normal PT the normal PT rules out an
    acquired prothrombin inhibitor.
  • Therefore, the most likely diagnosis is an
    acquired inhibitor against factor VIII.

36
Case 5
  • Acquired inhibitors against factor VIII are
    uncommon. They can occur post partum or in
    association with lymphoproliferative diseases or
    autoimmune disorders. They also can occur without
    any associated condition.
  • Patients frequently present with massive
    bleeding.
  • Treatment is aimed at 1) bypassing the inhibitor
    by using factor VIIa, porcine factor VIII
    (provided that the inhibitor does not
    cross-react), or activated prothrombin complex
    concentrates and 2) lowering the antibody levels
    using plasmapheresis or immunosuppression.
  • Postpartum factor VIII inhibitors will often
    resolve spontaneously.

37
Case 6
  • A previously healthy 50-year-old man is admitted
    to the hospital with a 2-week history of fatigue
    and dyspnea.
  • He is currently taking propranolol, clonidine,
    and ranitidine for hyper-tension and peptic
    ulcer.
  • Physical examination, including blood pressure,
    is normal. He is mentally alert.

38
Case 6
  • Laboratory evaluation
  • Hgb 4.6 g/dL, Hct 14, WBC 10,800/?L, normal
    differential, Plts 21K.
  • Cre 0.8 mg/dL, T bili 2.8 mg/dL, Dbili 0.4 mg/dl,
    LDH 2463 U/L.
  • Examination of the blood smear shows fragmented
    erythrocytes and polychromatophilia.
  • The direct antiglobulin test is negative.
  • Coagulation studies (prothrombin time, activated
    partial thromboplastin time, and fibrinogen) are
    normal.

39
Case 6
  • What is the most appropriate management for this
    patient?
  • (A) Suspect TTP and initiate plasma exchange
    therapy.
  • (B) Suspect autoimmune thrombocytopenia and
    hemolytic anemia (Evans syndrome), and initiate
    prednisone treatment, 1 mg/kg per day.
  • (C) Suspect disseminated carcinoma, and initiate
    a workup that includes chest radiographs, bone
    marrow examination, and abdominal CT scan.
  • (D) Suspect drug-induced thrombocytopenia and
    hemolytic anemia, and discontinue the three
    medications he is taking.
  • (E) Suspect pernicious anemia, and do a bone
    marrow evaluation to confirm megaloblastic
    hematopoiesis.

40
Case 6
  • The answer is A - even though the patient does
    not have renal manifestations, neurologic
    symptoms, or fever, he does have microangiopathic
    hemolytic anemia and thrombocytopenia. Moreover,
    he is not in DIC, since his PT/PTT are normal.
    Recognize that not every patient with TTP will
    have the pentad at the time of presentation,
    though many will have all symptoms--shortly
    before dying!!

41
Case 7
  • A 65-year-old man underwent bilateral knee
    replacement surgery 5 days ago.
  • His mobilization has been slow because of
    persistent pain, and he is only now getting up in
    a chair.
  • Because of a previous history of acute proximal
    venous thrombosis complicating a cholecystectomy
    8 years ago, he has been given subcutaneous
    heparin three times daily in doses sufficient to
    achieve an activated partial thromboplastin time
    of 35 sec.

42
Case 7
  • There are no clinical signs of fresh hemorrhage
    although ecchymoses and hematomas from the first
    ICU day are still evident.
  • There are no signs of hemorrhage from mucous
    membranes, needle stick sites, or tubes.
  • The hemoglobin is 12.5 Gm/dl, the white blood
    count is 4,500/ micro liter, no fragmented cells
    are seen, no platelet clumping is noted on the
    peripheral blood smear, the BUN is 35, the
    creatinine 1.6, the LDH is 180 (normal 84 - 197).

43
Case 7
  • The most likely cause of the thrombocytopenia is
  • (A) Ranitidine
  • (B) Sepsis
  • (C) Heparin
  • (D) Crystalloid given during surgery
  • (E) None of the above

44
Case 7
  • The best management approach for the patient is
  • (A) Stop the ranitidine because it is the cause
    of the thrombocytopenia.
  • (B) Obtain blood cultures, and culture any
    drainage from the left knee incision before
    starting antibiotic therapy.
  • (C) Stop the heparin.
  • (D) Send serum and plasma for studies to exclude
    heparin-induced thrombocytopenia continue the
    heparin until the results are available.
  • (E) Continue heparin, but follow the platelet
    count daily and give platelet transfusions if
    the count falls below 20,000/?L.

45
Case 7
  • The answers are c) and c). This patient has HIT,
    unless proven otherwise. Need to stop the
    heparin--may need to start alternate
    anticoagulation. Remember that 30-50 of pts
    with HIT will go on to develop thrombosis, so
    stop the heparin, then send off tests!!
  • Ranitidine is blamed too much for
    thrombocytopenia, but its easy enough to stop.

46
Case 8
  • You are asked to provide a consultation for a 22
    year old man with thrombocytopenia.
  • He was admitted to the ICU following a motorcycle
    accident in which he sustained head trauma, a
    ruptured spleen, two fractured ribs, and a
    fractured pelvis.
  • On the fourth ICU day the platelet count is
    reported to be 2,000

47
Case 8
  • Which of the following is the most likely
    diagnosis?
  • A. Disseminated intravascular coagulation
  • B. Post-splenectomy thrombocytopenia.
  • C. Thrombotic thrombocytopenic purpura
  • D. Pseudothrombocytopenia.

48
Case 8
  • Remember that multiple trauma predisposes to DIC.
    The answer is A
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