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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