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Hematology Case Studies

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HEMATOLOGY CASE STUDIES
58
CASE STUDY
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    CASE STUDY

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    The case.

    Elevated temperature

    Possible insect bite

    Small bite wound

    Necrosis was not evident

    Diffuse erythematous rash

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    PRESCRIPTION.

    40 ml of liquid acetaminophen Tylenol) and

    10 days of cephalexin Keflex)

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    A secretion from the lesion was

    subjected to culture, but there was no

    growth after 72 hours. Blood was drawn

    for cultures, which were reported as

    negative after 5 days.

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    Four days later.

    Unable to walk

    complete blood count,

    urinalysis, urine and sputum

    cultures, and chemistry

    profile

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    Laboratory

    Data

    Patient Reference

    WBC (x 109/L) 5.9 4.5 - 11

    RBC (x 1012/L) 1.14 4.35.9

    Hb (g/dl) 3.8 13.916.3

    Hct (%) 10 39 - 55

    Results.

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    Platelet count Normal range

    PBS Normocytic, normochromic, Few

    spherocytes, increase inneutrophilic bands, few early WBCs,

    3 nucleated RBCs

    Urinalysis 3+ blood

    Urine and sputum cultures negative

    chemistry profile elevated levels of total bilirubin,

    alkaline phosphatase, lactate

    dehydrogenase, and aspartate

    aminotransferase

    direct antiglobulin test (DAT) positiveindirect antiglobulin test negative

    eluate Negative

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    Guide questions.

    Is this type of anemia caused by

    intracorpuscular or

    extracorpuscular defects?

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    Answer.

    extracorposcular defects

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    What does a positive result of the

    DAT imply?

    Guide questions.

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    Answer.

    A positive DAT or Direct Antiglobulin

    Test which is a confirmatory of an immune

    hemolytic anemia implies that the patient

    has antibodies, a complement or both are

    present on the RBC surface.

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    What mechanisms can lead to the

    development of drug-related

    antibodies and drug-induced

    immune hemolytic anemia?

    Guide questions.

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    Answer.

    The hapten or drug adsorption mechanism

    The immune complex or innocent

    bystander mechanism

    Non-immune protein adsorption mechanism

    -methyldopa or autoimmune unknown)

    mechanism

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    Describe the mechanism that is

    most probable cause of this

    patients anemia

    Guide questions.

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    Answer.

    Hapten Drug Adsorption) Mechanism is the most probable cause.

    It happens when a hapten,a low molecular weight substance that rarely

    stimulates antibody production, is involved in drug induced hemolytic

    anemia.

    This is caused by drugs such as penicillin acetaminophen) which was

    administered to the patient.

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    The significantly high values of of total bilirubin,

    alkaline phosphatase, lactate dehydrogenase, and

    aspartate aminotransferase all indicate a damage to

    the liver.

    Answer.

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    What is the treatment of choice?

    Guide questions.

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    Answer.

    Removal of the offending drug

    ordinarily reverses the hemolytic

    process

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    Diagnosis: Drug Induced

    Hemolytic Anemia.

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    A 24-year old woman from Zaire

    was brought to the emergencydepartment because of periodic fever,

    chills, night sweats and fatigue. Her

    laboratory data revealed the following

    data:

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    Hematocrit: 0.35 L/L

    Reticulocytes: 275 x 109/L

    WBC Count: 11 x 109/L

    Thin Smear:

    slight variation in diameter of RBCs

    inclusions noted in the RBCs

    diffusely basophilic RBCs

    Thick film:

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    Based upon the observations made on

    the blood smear, one can conclude that thepatient suffers from parasitism. Becauseringforms of young trophozoiteswere seen on

    the blood smear.

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    Upon the examination of the thick film,there are numerous large number of ringforms in the red blood cells. The infectedcells have highly irregular surface defect.This may be produced by the intracellular

    growth of the parasite or it could representthe site of parasite entry. This

    phenomenon is known to occur in simianmalaria, the pitting of parasites from aninfected cell.

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    The diagnosis: the patient haveM L RI caused by lasmodium falciparum .

    One of the concrete evidence was the presenceof rings forms in the red blood cells in both films.

    The erythrocyte in P.falciparum infection isnot enlarged. There is the presence of uersdot and may be a few reddish staining dots. Thepresence of extremely numerous rings and no

    other stages are seen strongly indicatesP.falciparum case.

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    CASE NO. 4

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    A 55 year old man sought a medical

    attention for the onset of chest

    pain. Physical examination revealed

    slight jaundice and splenomegaly. The

    past medical history included

    gallstones, and there was a family

    history of anemia.

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    A complete blood count

    (CBC) yielded the following

    results:

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    Laboratory

    Data

    Patient Reference

    Range

    WBC (x 109/L) 13.4 4.5-11

    RBC (x 1012/L) 4.28 4.3-5.9

    Hemoglobin (g/dL) 11.7 13.9-16.3

    Hematocrit (%) 32.5 39-55

    MCV (fL) 76 80-100

    MCH (pg) 27.3 25.4-34.6

    MCHC (g/dL) 36 31-37

    RDW (%) 22.9 11.5-13.5

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    peripheral blood smear

    revealed:

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    slight anisocytosisslight polychromasia

    several dark, round

    microspherocytes lackingcentral pallor

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    The platelet count andplatelet distribution on the

    smear were normal.

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    QUESTIONS

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    Q1

    From the data given, what is

    your initial diagnostic

    assessment of the anemia?

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    Q1

    From the data given, what is

    your initial diagnostic

    assessment of the anemia?

    Hereditary spherocytosis

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    Q2

    What additional laboratory testswould be of value in establishing

    the diagnosis, and what

    abnormalities in these tests would

    be expected in confirming yourimpression?

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    Q2

    What additional laboratory tests would be of value in establishing the

    diagnosis, and what abnormalities in these tests would be expected in

    confirming your impression?

    Mearuement of bilirubin An increased

    bilirubin level would be expected

    Reticulocyte count An increased

    reticulocyte count

    Measurement of haptoglobin A decreased

    or absence of haptoglobin

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    Q2What additional laboratory tests would be of value in establishing the

    diagnosis, and what abnormalities in these tests would be expected inconfirming your impression?

    Osmotic fragility test An increased

    Osmotic fragility test

    Chromium labelled RBC test Abnormal (15

    days)

    Fecal urobilinogen Presence of

    urobilinogen because of destruction of

    ertythrocytes

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    Q2What additional laboratory tests would be of value in establishing the

    diagnosis, and what abnormalities in these tests would be expected inconfirming your impression?

    Lactate dehydrogenase (LDH) Test An

    increased LDH level

    Coombs test A negative Coombs test

    Erythorocyte autohemolysis test An

    increased autohemolysis test andcorrected by the addition of glucose.

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    Q3

    What is the cause of this typeof anemia?

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    Q3

    What is the cause of this type of anemia?

    Hereditary spherocytosis is due to the

    presence of an autosomal dominant mutation.

    This mutation caused an abnormal red blood

    cell membrane (abnormal or lack of spectrin).

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    This disorder is most common in

    people of Northern Europe

    descent, but it has been foundin all races.

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    An 18-year old African American woman was

    seen in the emergency department for fever and

    abdominal pain. The following results were

    obtained on a blood count:

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    WBC count: 11.9 x 109/L - increased

    RBC count: 3,67 x 1012/L - decreased

    Hgb: 10.9 g/dL decreased

    Hct: 32.5% (0.325L/L) decreased

    Platelet count : 410 x 109/L - normal RDW : 19.5% - increased

    Segmented neutrophils : 75% - normal

    Lymphocytes : 18% - normal

    Monocytes : 3% - normal Eosinophils : 3% - normal

    Basophils : 1% - normal

    Reticulocyte count: 3.1% - increased

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    1.Select the confirmatory tests that should be

    performed and the expected results.

    - Confirmatory test should be performed arehemoglobin solubility test and cirtate agar test,

    hemoglobin C I s separated from hemoglobin A, O, andE as a result of mode of migration; hemoglobin Cmigrates more toward the anode, whereas hemoglobins

    A, O, and E migrate toward the cathode. Likewise,

    hemoglobin S migrate toward the cathode. Likewise,hemoglobin S migrates anodally, wheraes hemoglobinsD and G migrate cathodally.

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    2. Describe the characteristic RBC morphologyon the peripheral blood film.

    - Characteristically, red blood cell that containcystallized aggregates of hemoglobin thatprotrude through the cell membrane are seen. Theyare irregular shaped cells which appear to contain

    mis-shapen crystals and increased target cells areseen.

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    3. Based on the electrophoresis and RBCmorphology results, what diagnosis issuggested?

    - On the basis of the ectrophoretic pattern,the daignosis of presence of hemoglobin SC

    can be made

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    4. If this patient were to marry a person of genotypeHbAS, what would be the expected frequency ofgenotypes for each of four children?

    - According to Mendelian law, the genotype can bedepicted by the following chart:

    A S

    S AS SS

    C AC SC

    25% Would be each genotype

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    Mild, chronic hemolytic anemia associated withvariable vaso-occlusive complications.

    Splenomegaly

    Hgb level usually 1113% g/dL

    Reticulocyte count: 3 5%

    Peripheral smear: few sickle cell, target cells. And intra

    erythrocyte free crystalline structures (may protrudefrom membrane, HbSC crystals)

    Citrate agar: C migrates, separation from S, E, O

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    Diagnostic Hematology by: Rodak, Bernadette F. Clinical Hemetolology: Correlations and Principles by:

    Steininger, Sheryl


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