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50 Cards in this Set

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Acute myelogenous leukemia (AML-M1)
t(9;22)
t(15;17)(q22lq12-21)
Retinoic acid receptor (17)
translocated to PML gene (15q); 90%
Acute promyelocytic leukemia (APML)
Chronic myelogenous leukemia (CML)
t(9;22)(q34;q11)
Breakpoint cluster region (bcr) on 22;
abl on 9; makes abnormal tyrosine kinase
MALT lymphoma
t(11;18)(q21;q21)
API2 and MLT
t(3;14)(q27;q32)
bcl-6/IgH
Diffuse large B-cell lymphoma
t(14;18)(q32;q21)
bcl-2 gene (18) translocated to Ig gene(14)
Follicular lymphoma (FL)
t(2;5)(p23;q35)
ALK and NPM
Anaplastic large cell lymphoma (ALCL)
Burkitt lymphoma
t(8;14)(q24;q32), t(2;8), and t(8;22)
c-myc gene (8) translocated to Ig gene
(14), k gene (2), or l gene (22)
t(11;14) involving BCL1 (cyclin D1) and IgH
Mantle cell lymphoma
An amino acid substitution of lysine for glutamic acid in position 6 of the b hemoglobin molecule is
characteristic of which Hb?
Hb C is characterized by a lysine substitution at position 6, whereas Hb S is a valine substitution. In Hb E
lysine is substituted for glutamic acid at position 26. The thalassemias are quantitative disorders of hemoglobin.
30 y/o Asian female is found to have a homozygous hemoglobinopathy. She has a hypochromic anemia
and splenomegaly. Which of the following is the most likely the etiology?
Hb E is frequently seen in Asian populations, and is characterized by a hypochromic anemia and
splenomegaly. A lysine is substituted for glutamic acid at position 26
When considering hemoglobin electrophoresis on cellulose acetate at pH 8.4, which of the following is the
correct order of Hb migration from the cathode towards the anode?
C S F A
In alkaline pH, hemoglobin is negatively charged. The most negatively charged hemoglobin will move the
fastest towards the anode (+ charge). The pneumonic A Fat Santa Claus:
anode to the cathode
low pH =FASC+
300 gram spleen, which was noted to have multiple blood filled
nodules. The cells of interest were positive for CD68 and CD31 but negative for CD34
Littoral Cell Angioma is a vascular lesion, which resembles splenic sinuses. Interestingly, the
cells express CD68 (histiocyte marker) and CD31 (endothelial marker), but they lack expression of CD34
(present in other vascular lesions).
spleen removed for “symptoms"; 1,000 grams
and had multiple small white nodules throughout the parenchyma. positive for CD20 but
negative for CD5, CD43 and CD10.
Marginal Zone Lymphoma (MZL)
there is more abundant cytoplasm than in CLL/SLL or MCL.

Second, there is more heterogeneity of the cell size and shape compared to
MCL or CLL/SLL. The cells are small to intermediate in size, and there may be plasmacytoid
differentiation.
Multiple vascular lesions were found in a 70 year old male with no other significant past
medical history. No other lesions in other organ were found.
Angiosarcoma of the Spleen
A calcified lesion was found incidentally in a 20 y/o. At surgery it was found to originate
from the 400 gram spleen.
Epithelial Cyst Cysts in the spleen are not common. If there is a true lining, then they are
considered true cysts. Epithelial cysts with squamous lining are thought to arise from metaplasia of a
mesothelial cyst.
A 24 y/o male with a history of short stature and renal failure (s/p renal transplant) presented
with multiple splenic abscesses. A splenectomy was performed
Cystinosis involving the Spleen Cystinosis is an autosomal recessive disorder that affects
approximately 400 individuals in the United States.
The classic finding in urine
cytology are hexagonal crystals.
B cell lymphoma
A patient is diagnosed with CLL/SLL. Which of the following are associated with a poor
prognosis:
Del 13q14 is a good prognostic marker in CLL/SLL.

Del 11q23 and trisomy 12 are associated
with a poor prognosis

CD38 and ZAP-70 expression are also associated with a more aggressive course.
acute leukemia
HHV-8 is associated with
HHV-8 is associated with multi-centric Castleman’s disease, PEL, and Kaposi’s sarcoma.
AML good prognosis?
t(8;21), inv(16), t(16;16), and t(15;17) are associated with a
relatively good prognosis.
Examination of a lymph node shows extensive infiltration by macrophages with foamy
cytoplasm. Staining with PAS, shows diastase resistant material. AFB stain is negative. Which of the
following answers would be the best diagnosis:
A. MAI
B. Syphilis
C. Rosai-Dorfman
D. Toxoplasmosis
E. Whipple’s disease
E. Whipple’s disease is caused by bacillary organisms (Tropheryma whippelii), which fill the
cytoplasm of histiocytes and stain with PAS, and are AFB negative. Test taking strategy. The following are
important “catch” phrases for the other answers: MAI  AFB + histiocytes, Syphilis  Plasma cells,
Roasi-Dorfman  Histiocytes with emperipolesis and perivascular plasma cells, and Toxoplasmosis 
monocytoid B-cells.
B-cell prolymphocytic leukemia is characterized by which of the following?
A. 1-10% prolymphocytes
B. 11-55% prolymphocytes
C. >55% prolymphocytes
D. > 75% prolymphocytes
E. None of the above
PLL is defined by >55% prolymphocytes. >10-55% is classified as CLL/PLL, and up to 10% is
tolerated in CLL.
AML-M0?
>90% myeloblasts of the non-erythroid cells, no Auer rods, and less than
3% positivity of myeloperoxidase (MPO) in the myeloblasts.
AML-M1?
AML-M1 is the FAB classification of AMLs without maturation. Meaning that <10% of the
non-erythroid cells exhibit maturation beyond the blast stage. There may be rare Auer rods, and >= 3%
positivity of myeloperoxidase (MPO) in the myeloblasts.
AML-M2?
AML-M2 is characterized by maturation of at least 10% of non-erythroid cells. This is in
contrast to AML-M1, which shows maturation in < than 10% of non-erythroid cells.
AML-M3
AML-M3 is also known as acute promyelocytic leukemia. It is defined by the t(15;17), and is
responsive to therapy with all-trans retinoic acid. This is an important diagnosis to make because it has a
high long-term cure rate, but can be very deadly in the acute stage due to DIC. DIC can be induced by
degranulation of the leukemic cells.
AML-M2 is characterized by all of the following EXCEPT:
AML-M2 shows greater than 10% differentiation of myeloid cells and is composed of <50%
erythroid cells. >= 3% of the cells will be myeloperoxidase positive (if less than 3% of the myeloblasts are
positive for myeloperoxidase, then it will be classified as AML-M0). These myeloblasts are usually positive
for CD13, CD33, and HLA-DR. The most frequent translocation associated with AML-M2 is t(8;21), which
is the AML1/ETO gene fusion (approximately 20% of cases).
What is the minimum percentage of blasts is required in the peripheral blood to meet the
criteria for a diagnosis of acute leukemia?E
20% blasts is the minimal requirement for the diagnosis of acute leukemia in both the bone
marrow and the peripheral blood.ïýïý
AML-M2 with basophilia are more often associated with which of the following
translocations?F
AML-M2 with basophilia is associated with both t(6;9) and t(12p).
In general AML-M2 is associated with a long-term remission, but this does not occur in patients with AMLM2
Baso.o
A 40 year old woman presents with an elevated WBC count (25K) with abnormal immature
cells noted by the hematology technologist (see case images). Flow cytometry demonstrated the cells to be
positive for CD13 and CD33 and negative for CD34 and HLA-DR.
A. B-ALL
B. AML-M0
C. AML-M2
D. AML-M4
E. APL
This case represents an AML-M3 (a.k.a. Acute Promyelocytic Leukemia, APL). It is
characterized usually by a lower presenting WBC count compared to other leukemias and exhibits maturity
just past the myeloblast stage, which is why CD34 and HLA-DR are often negative. Note the intense
staining of the blasts with myeloperoxidase.
how long do reticulocytes persist
In a normal patient reticulocytes persist for ~24 hours. In cases of anemia, the immature RBCs may be
released early from the bone marrow, and therefore circulate for a longer period of time. This is why there are
correction factors for reticulocyte response in anemic patients based on the hematocrit.
A 32 y/o female presents with fatigue. Laboratory studies show a low RBC count, decreased
hemoglobin/Hematocrit, decreased MCV, increased RDW, and an iron saturation of 17%. What is the best
diagnosis, and which piece of information is most helpful in distinguishing it from other possible answers
listed?
a. Iron deficiency anemia, iron saturation
b. Thalassemia, RDW
c. Iron deficiency, MCV
d. Thalassemia, RBC count
e. Iron deficiency anemia, increased RDW
E. Iron deficiency anemia and thalassemia are both microcytic hypochromic anemias. They differ in several
ways. Thalassemia usually has an increased RBC count and normal RDW, whereas iron deficiency anemia usually
has a decreased RBC count and increased RDW. Iron saturation can cause confusion in that it can be decreased in
thalassemia (although usually >15%), which results in some overlap with iron deficiency
GPIb receptor
GPIb receptor on the platelet is responsible for initial adhesion of the platelet to the vessel wall
via vWF as a bridge, which binds to exposed collagen.
GPIIb/IIIa receptor
GPIIb/IIIa receptor mediates aggregation of platelets with fibrinogen serving as the bridge
between the GPIIb/IIIa receptors
Moderate hemophilia A is characterized by which of the following approximated factor VIII levels
1-5%
Adhesion of platelets is mediated through
GPIIb/IIIa receptor on platelets is responsible for aggregation. The GPIIb/IIIa receptor binds
with fibrinogen to create a platelet plug. GPIb, collagen, and von Willebrand's factor all work in concert
together to cause adhesion of the platelet to the vessel wall. When the endothelium of the vessel is disrupted,
collagen is exposed which binds to von Willebrand's factor. Von Willebrand's factor serves as a bridge
between the collagen and the GPIb receptor on the platelet.
The PFA-100 analyzer has replaced the bleeding time at many institutions. This test utilizes two
tubes for analysis. One tube contains collagen and ADP, while the other tube has collagen
epinephrine. In a patient who has a prolonged bleeding time, which of the following would have a
normal clotting time in the collagen and ADP tube but be prolonged and the collagen epinephrine
tube?
Aspirin results in a thromboxane deficiency. In the PFA-100 analyzer, a thromboxane
deficiency is overcome by the abundance of ADP. Therefore this test can be helpful in separating storage
pool defects, which include aspirin, from other possible at realities. That being said, bleeding times and the
PFA-100 are still not very sensitive or specific tests.
treating a patient with hemophilia A:
FFP contains very little factor VIII needed to increase the patient’s levels. Cryoprecipitate is
rich in vWF and Factor VIII (remember they travel together). DDAVP releases factor VIII and vWF from
endothelial cells.
A patient underwent cardiac bypass surgery and was having an uneventful post-operative course. On
POD #7, the patient developed an ischemic leg. The intern asks your help in diagnosis, and also
informs you that his platelet count has recently dropped. The most likely etiology is:
Heparin induced thrombocytopenia (HIT) usually develops approximately 5-10 after starting
treatment with heparin (time period can be shorter for patients with previous exposure). In cases of HIT with
significant risk of thrombosis the platelet count usually drops >50%. HIT results from antibodies against
heparin bound to PF4 on platelets. The platelet count usually recovers in 2-5 days after discontinuing
heparin.
Heparin induced thrombocytopenia
Heparin antibodydiagnosing the patient
patient who is known to have von Willebrand’s disease (vWD) has a normal vWF antigen and
decreased levels of Factor VIII. What is the most likely vWD subtype in this patient
vWD type 2N (Normandy) is characterized by normal amounts of vWF, which interact with
platelets in a normal fashion. They have a mutation in the region that binds with Factor VIII, which results
in decreased affinity. Factor VIII is degraded in the plasma approximately five time faster than when
protected by vWF. Platelet type, pseudo-von Willebrand’s disease looks identical to vWD type 2B, except
the mutation is a gain of function in the GPIb receptor where as vWD type 2B is a gain of function in the
vWF.
A patient is suspected of having Bernard-Soulier disease. If the patient has this disease, they would
show response to all of the following during platelet testing EXCEPT
Ristocetin
Bernard-Soulier disease is defined by deficiency of the GpIb receptor on the platelet. GpIb is
responsible for platelet adhesion, which is stimulated only by ristocetin in platelet testing.
A young patient is diagnosed with a pulmonary embolism, and is found to have a large DVT.
No acquired risk factors for hypercoagability are found, and genetic etiologies are suspected. The patient is
still in the ER and has not yet received anticoagulation. Which of the following tests can be performed to
evaluate possible hypercoaguable states:
A. PT20210
B. Protein C and S
C. Factor V Leiden
D. A and C are correct
D. In the acute state of a thrombosis, only genetic based tests should be done. Plasma tests for
“levels” are not helpful in the setting of an acute thrombus or anticoagulation because they will be affected.
These include: protein C and S, antithrombin III, factor VIII. Genetic tests including Factor V Leiden and
PT20210 (PCR based testing) can both be performed acutely or while on anticoagulation therapy.
Of extracellular matrix constituents, which is the most important pro-thrombotic component?
Collagen is the most important pro-thrombotic component of the extra cellular matrix. Von
Willebrand’s factor acts as a bridge between collagen and platelets.
A 46 y/o woman is found to have a prolonged aPTT with a normal PT. A mixing study was
performed, and the aPTT corrected into the high normal range. Clinically, the patient does not have any
evidence of bleeding or bleeding tendencies. Further questioning reveals the patient has been noted to have a
prolonged aPTT during a routine physical exam 5 years ago. He was referred to a hematologist at that time
who told him he was fine and not to worry. Based on these findings, which of the following is the most
likely etiology of the patients prolonged aPTT?
In vitro factor XII is required to bind to glass beads to activate the intrinsic clotting pathway.
This factor is not required to activate the clotting cascade in vivo, and patients with deficiencies of factor
XII, high molecular weight kininogen, or prekallikrein do not have a bleeding diathesis clinically. A mild
hemophilia A might be a reasonable answer, but the patient is a woman, and hemophilia A follows an Xlinked
inheritance pattern. Lupus anticoagulant and factor V inhibitors would not result in a corrected
mixing study.
An acquired inhibitor to factor X can be caused by which of the following?
Amyloidosis is a cause of an acquired inhibitor to factor X. Hemophilia A patients may
develop Factor VIII inhibitors, and Hemophilia B patients may develop inhibitors to factor IX. Use of
bovine thrombin in vascular surgery is associated with development of factor V inhibitors.
Heparin induced thrombocytopenia is caused by heparin interacting with:
Platelet factor 4
Positive stains: bright surface immunoglobulin, CD5, CD19, CD20 (bright), CD22, FMC7
Molecular: 14q+ is most common; cytogenetic abnormalities are complex, suggesting clonal evaluation and genomic instability
Prolymphocytic leukemia