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Factoids for DHM block part 2 Flashcards

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1
Q

What general histological features would you look
for to make a diagnosis of lymphoma?

A

•
Loss of normal architecture
•
Infiltration by
–
Abnormal structures that may be a clue to cell of origin
(e.g. follicular lymphoma)
–
Diffuse sheets of similar cells or mixed population
–
There may be abnormal fibrosis or abnormal
vasculature reflecting abnormal cytokine production

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2
Q

Why do young children get acute lymphoblastic leukemia (ALL)?

A
  1. They have developing B lymphocytes in the marrow and T lymphocytes in the thymus
  2. Some of the initiating molecular events occur in utero
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3
Q

Typical presentation of B-ALL

A

Precursor B lymphoblastic
leukemia/lymphoma (B
-
ALL)
•
Typical presentation
–
Fatigue, bleeding: marrow failure
–
Bone pain: rapid growth of tumor in marrow
–
Headache, vomiting, nerve involvement:
may indicate CNS involvement
–
Variable lymph node, organ infiltration
•
Hence “lymphoma”

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4
Q

Diagnosis?

A

Precursor T lymphoblasticleukemia/lymphoma (T-ALL)

Precursor T lymphoblastic leukemia/lymphoma (T - ALL) • Presentation
: typically a mediastinal mass in a
boy
–
Can also present similar to B
-
ALL
•
Morphology
–
Mass has sheets of immature blasts
–
Blood and marrow variably involved
–
Similar morphology to B
-
ALL
•
Major diagnostic immunophenotypic markers
–
Typically cytoplasmic CD3+,
tdt+,
with variable
expression of CD1a, CD10+, and T cell markers
(CD2, 4, 8, 7, 5)
–
Frequent aberrant myeloid or B cell marker
expression

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5
Q

CLL prognostication

A

–
Poor prognosis:
•
ZAP70+ (about 50% of cases)
–
A T cell lineage cytoplasmic tyrosine kinase that transduces the
T cell receptor signal
–
Not expressed in normal B cells
•
Unmutated immunoglobulin genes
–
Pre
-
germinal center cells
–
Usually ZAP70+
•
Cytogenetics
–
del(17p) p53 locus
–
del(11q22)
»
Ataxia telangiectasia mutant gene (ATM) locus
–

Good prognosis
•
“Watch and wait”
–
May not progress for 5 to 10 years
•
del(13q),
•
ZAP70 -
•
Mutated immunoglobulin genes.

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6
Q

What is expressed in Follicular lymphoma to use as diagnostic?

A

This is the only lymphoma where Bcl2
expression is evidence of malignancy,
because the cells of origin in other
lymphomas normally express Bcl2

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7
Q

What does follicular lymphoma often transform into?

A

Transformation to DLBCL common
•
30- 50% of cases eventually transform
•
Cases that acquire a C-myc
IgH
translocation are
called “double hit” lymphomas

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8
Q

Burkitt Lymphoma

A

A rapidly growing mass in characteristic
settings with a c -myc **(t(8;14)) **translocation, which can be cured with very aggressive chemotherapy, but otherwise lethal.

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9
Q

Burkitt lymphoma diagnostic features

A

Immunophenotype: germinal center B cell
markers (CD20, CD10, bcl6)
•
Almost 100% Ki-67+ (proliferation marker)
•
Bcl2 is not expressed
•
c-myc translocation (usually t(8;14))–
Result of aberrant somatic hypermutation
(endemic) or class switch recombination
(sporadic) in the germinal center

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10
Q

Most important predictor in plasma myeloma?

A

beta-2 -microglobulin
•Released from the tumor cells
•Most important predictor

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11
Q

Plays an important role in pathogenesis of Multiple myeloma?

A

**IL-6:paracrine growth factor produced by marrow stromal cells. **

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12
Q

Waldenstrom’s macroglobulinemia

A

Hyperviscosity due to elevated IgM
•
Homogeneous band (M-spike) on serum
electrophoresis produced by neoplastic clone
•
Almost always due to LPL, rarely other lymphomas
•
Molecular hallmark: Activating mutations of MYD88
L265P (an intermediate in toll-like receptor
signaling pathway) in 90% of cases

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13
Q

Lymphoplasmacytic lymphoma (LPL)

A

Symptoms related to
hyperviscosity
–
Visual impairment,
cerebrovascular
accidents (stroke)
–
Raynaud’s
phenomenon due to
•
cold agglutinins
–
Antibodies to red cells that bind below 37
o
C
•
Cryoglobulins
–
Type I (one component only)
-
IgM
antibodies that precipitate in the cold
•
Symptoms related to tumor infiltration
–
Bone marrow infiltration crowds out normal hematopoiesis
•
Does not produce lytic lesions
–
Some have organomegaly, lymphadenopathy
•
Symptoms related to anemia
–
Marrow infiltration
–
Does not produce lytic lesions
–
Hemolysis
•
Bleeding
•
Paraprotein interferes with platelet functions and coagulation factors

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14
Q

In B cell lymphomas, why is there frequently
translocation of an onocogene to chromosome 14q32 ?

A

As an early event in cell transformation, an
oncogene
is translocated to the
immunoglobulin heavy chain locus on
chr 14q32. The
oncogene
gets deregulated
( overexpressed ) due to the new proximity of
a strong immunoglobulin locus enhancer
element

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15
Q

Splenomegaly and pancytopenia

Male predominance

A

Hairy Cell Leukemia

Morphology: Medium sized cells with
projections.
Usually leukopenia

Activating BRAF V600E mutations in essentially all cases

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16
Q

What other tumors have BRAF mutations?

A

Thyroid carcinomas
–
Melanoma
–
Some colon cancers
–
Hyperplastic polyps and serrated adenomas
–
Langerhans
’ cell histiocytosis

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17
Q

Describe positive selection in the thymus.

A

Thymic Cortex
(positive selection of T cells)
•
Numerous immature T
-
cells/
thymocytes
(many
co
-
express
CD4 and CD8)
Note: Precursor T lymphoblastic lymphoma/leukemia is
the neoplastic analogue to the immature T
-
cell
•
Scattered, individual cortical epithelial cells
present self antigens in association with MHC
-
I
and
II
molecules
–
T
-
cells that appropriately recognize self MHC
molecules receive signals for survival

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18
Q

Describe negative selection

A

Thymic medulla
(negative selection of T cells)
•
Final stages of maturation: CD4+
or
CD8+
•
Medullary epithelial cells also present
antigens and MHC molecules to T
-
cells
–
T
-
cells with too high an affinity for self
antigens
→ negative selection/apoptosis

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19
Q

Core binding factor

A

•
t(8;21) and inv(16) AMLs have
translocations of the core binding factor
(CBF) α and β subunits
•
Called core binding factor AMLs
•
CBF is a transcription factor active in
granulocyte maturation

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20
Q

Why would you expect a DNA demethylating
agent to work in treatment of MDS or AML?

A

Because tumor suppressor genes are
inactivated in AML by abnormal
methylation due to mutations in epigenetic
modifiers (e.g. DNAmt3a, Tet2, IDH)

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21
Q

How would you confirm diagnosis of CLL?

For prognostic?

A

Flow cytometric immunophenotyping
•
All cells should express the same Ig light chain
isotype
•
Should have aberrant coexpression of CD5 and
CD23 on the malignant B cells.

For prognostic purposes:

CD38 and Zap70 expression
–
Cytogenetic analysis, including FISH
–
Immunoglobulin sequencing to determine if
pre
-
or post germinal center (Ig
hypermutation)

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22
Q

For CLL, What histological pattern do expect to find in the lymph nodes and spleen?

A

–
Lymph nodes
•
Diffuse effacement with scattered proliferation
centers (pseudofollicles)
–
Spleen
•
Widespread white pulp involvement spilling into
the red pulp.

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23
Q

Expression of this enzyme which is
involved in n
-
nucleotide addition, identifies
a lymphoid neoplasm as acute leukemia.

A

What is terminal deoxynucleotidyl
transferase or TDT?

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24
Q

This laboratory technique, performed on
live cells, allows for the assessment of
light chain restriction on the surface of B
cells

A

flow cytometry

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25
This myeloid disorder can arise de novo, as a result of progression of an underlying disease or after chemotherapy and is classified based on cytogenetics and for prognostic and therapeutic purposes.
AML
26
This special category of low grade myelodysplastic syndrome occurs in older females and presents with anemia and increased platelets
5q minus syndrome
27
This condition results from implantation of splenic tissue on serosal tissue within the abdomen after traumatic splenic rupture
splenosis
28
In Hand-Schuller-Christian syndrome, diabetes insipidus results from compression of the posterior pituitary by what neoplasm?
What is Langerhans cell histiocytosis/eosinophilic granuloma/histiocytosis X
29
B. pertussis Vaccine
• Current**_ acellular_** vaccine has fewer side effects (fever, pain; rare convulsions & shock-like state) than old whole cell vaccine •Given as part of DTaP vaccine at 2, 4, 6, 18 months and 4 years of age •Not given after 6 years of age
30
Diphtheria Vaccine
Toxoid is formaldehyde inactivated toxin •Administered with alum as adjuvant •Given as part of DTaP vaccine at 2, 4, 6, 18 months and 4 years of age and as booster (dT) containing less diphtheria toxoid at 15 years of age.
31
Tetanus Vaccine
* Toxoid is formaldehyde inactivated toxin * Administered with alum as adjuvant * Given as part of DTaP vaccine at 2, 4, 6, 18 months and 4 years of age and as booster at 15 years of age and every 10 years thereafter.
32
Oral (Sabin)
(Sabin - live attenuated) = OPV – Produces mucosal (IgA), as well as IgG Ab – Prevents carriage in GI tract – Secondary spread – can cause polio in rare (immunocompromised) individuals
33
Injected Polio Vaccine
Injected (Salk-killed) = IPV – **Produces IgG only,** does not prevent carriage; no secondary spread, but SAFE – Only polio vaccine used now in the USA – Given at 2, 4, and 6 - 18 months and again at 4 - 6 years of age
34
Measles Vaccine
Measles Vaccine •Single strain •Live, attenuated viral vaccine •Given as part of measles, mumps, rubella (MMR) •Give at 15 months (earlier, and residual maternal antibody might neutralize) •Booster at 6 or 12 years of age **•DO NOT give live vaccine to immunocompromised patients!**
35
Rubella Vaccine
Rubella Vaccine * Single strain * Live, attenuated viral vaccine * Given as part of measles, mumps,rubella (MMR) * Give at 15 months (earlier, and residual maternal antibody might neutralize) * Booster recommended at 6 or 12 years of age * DO NOT give live vaccine to immunocompromised patients! * DO NOT immunize pregnant woman (3 month residual virus)
36
Mumps Vaccine
* Single strain * Live, attenuated viral vaccine * Given as part of measles, mumps,rubella (MMR) * Give at 15 months (earlier, and residual maternal antibody might neutralize) * Booster recommended at 6 or 12 years of age * DO NOT give live vaccine to immunocompromised patients!
37
Hib Vaccine
•**Hemophilus influenzae_ type b capsular polysaccharide_**bound to **_protein carrier (diphtheria toxoid, tetanus toxoid, or meningococcal outer membrane protein (OMP)_** * Given at 2, 4, 6, and 12-15 months of age * Does not protect against unencapsulated strains, or other strain types, which cause otitis media
38
Hepatitis B in Neonates
Hepatitis B • While only ~10% ofsymptomatic adults infected with HepatitisB virus become chroniccarriers, ~50% of neonates do! Results: –Fulminant hepatitis –Primary liver cancer –Cirrhosis –Chronic carriage •Virus is transmitted bymaternofetal transfusion at birth
39
Hepatitis B Vaccine
Hepatitis B Vaccine •Purified, recombinant Hepatitis B surfaceantigen •Given at birth, 1-4 months, and 6-18 months; catch -up later if needed •Given along with passive immunization (Hepatitis B immune globulin-HBIG) if mother is known to be HBsAg positive
40
Is VZV serious?
VZV can crossplacenta-serious infection (35%v mortality) •Risk of post-infectious sequalle –Reye’s Syndrome (fatal) –Shingles
41
Varicella Zoster Virus Vaccine
Live, attenuated viral vaccine •Give at 15 months (earlier, and residual maternal antibody might neutralize) **•DO NOT give live vaccine to immunocompromised patients!** •VZV immune globulin available for high risk patients
42
Streptococcus pneumoniae Vaccine
–13 valent conjugate now recommended for all –Given at 2, 4, 6, and 12 to 16 months of age –23-valent polysaccharide given to high risk: sickle cell disease, asplenia, elderly, etc.
43
Passive Immunotherapy: Transfer of Immunoglobulins
* Hepatitis A * Hepatitis B (HBIG) * Measles * Rabies * Varicella Zoster (VZIG)(Pregnancy) * Tetanus (TIG) * Passive-active combination used in rabies, tetanus, Hepatitis B
44
Osteoblasts
* Located on bone spicule surfaces * Synthesize, transport and arrange matrix proteins * Initiate mineralization * Receptors for PTH,VitD,and estrogen * Become osteocytes once surrounded by matrix
45
Osteocytes
* Most numerous bone cell * Surrounded by bone * Connected through canaliculi * Gap junctions transmit membrane potentials * Control of serum calcium and phosphorus * Detect mechanical forces
46
Osteoclasts
* Bone resorption * Monocyte/macrophage lineage * Multinucleate(6–12 nuclei) * Howship lacunae–resorption pits * Sealed extracellular space = secondary lysosome
47
Non - neoplastic Bone Disease
* Non-neoplastic Bone Disease * Developmental Abnormalities * Osteoporosis * Paget Disease * Mineralization Disorders * Fractures * Osteonecrosis
48
Thanatophoric Dwarfism
Usually lethal 1 in 20 -60K live births Missense mutationFGFR Features: –Micromelic limb shortening –Bell-shaped abdomen –Brain abnormalities –Macrocephaly with frontal bossing –Small chest cavity –respiratory insufficiency –death at birth orsoon after
49
Osteopetrosis
Marble Bone Disease (Albers -Schonberg)Osteoclast dysfunction –Abnormally dense & brittle bones –Erlenmeyer flask deformity –Absent medulla, woven bone Fractures, anemia, hepatosplenomegaly Tx–Bone marrow transplant
50
Disease?
**Paget Disease** Osteitisdeformans * Osteoclastover-function * ? Slow virus infection * Overlapping andrepetitive phases * **Mosaic bone**
51
Fracture Healing
**Fracture Healing Procallus** –Soft tissue callus –Hematoma, fibrin mesh,fibroblast, capillaries **Bony callus** –Woven bone, cartilage, enchondral ossification **Callus remodeling,** –Restore medullary canal –Re-establish normal shape and outline –Reconstitue normal structure
52
Osteoma
Bosselated , sessileMostly skull orfacial bonesUsually solitary Middle-aged adults Gardner syndrome –Multiple osteomas , GI adenomas, epidermal cysts, fibromatosis Woven and lamellar bone –Resembles reactive bone Usually slow growth –Treatment for local mass effects –No malignant transformation
53
Enchondroma
Benign tumors of hyaline cartilage Most common intraosseous cartilage tumor Most frequent in the 20’s to 40’s Usually solitary, metaphysis of tubular bones, especially hands and feet –Multiple in hereditary syndromes Rests of growth plate cartilage; mainly enchondral bones
54
Enchondroma size and apearance?
Less than 3 cm Gray-blue, translucent and nodular Nodules of hyaline cartilage and benign chondrocytes Periphery-enchondral ossification Center often calcifies
55
Chondroblastoma
* Rare benign tumor * \<1% of bone tumors * Teen-agers * Male-to-female 2:1 * Epiphysis andapophysis * –Most around the knee
56
Chondrosarcoma
Second most common primary malignant tumor Half as frequent as osteosarcoma Patient in their 40’s or older Variants more common in younger patients – Clear cell and mesenchymal Male - to - female 2:1 Central skeleton – pelvis, shoulder, ribs Clear cell variant – epiphyses of long bone Rare in distal extremities
57
Ewing Sarcoma and PNET
* 6-10% of primary bone sarcomas * Second most common bone sarcoma in * children (after osteosarcoma) * Youngest** **average age (10-15) of bone sarcomas * Caucasian \>\>\> African-American * t(11;22)(q24;q12)–85%
58
What germ layer are hematopoietic cells derived from?
Mesoderm
59
Embryologically, the hematopoietic stem cell lineage is most closely related to what other cell type?
Endothelial cell (Hemangioblast is the common progenitor in yolk sac island, similar origin from wall of aorta in definitive hematopoiesis.
60
Which of the following molecules are intrinsically kinases? 1. Notch 2. Jak 3. Stat 4. G-CSF receptor 5. C-kit 6. FLT3
**Jak (Janus Kinase) C-kit FLT3**
61
What is the function of Colony stimulating factors?
Act to promote growth, block apoptosis and promote maturation
62
What is the most common cause of iron deficiency anemia in the US?
Chronic blood loss
63
A person has beta thalassemia with no expression at all of one of the beta-globin alleles due to a point mutation at a splice junction. What will you expect clinically and in the CBC?
Normal or near normal Hb. Microcytosis •Thal minor/trait
64
A person has 3 alpha chains deleted. What happens to the excess beta chains?
``` Beta tetramers (HbH forms and precipitates-golf ball cells) •What is the phenotype? •Thalassemia intermedia ```
65
A previously normal person has anemia with spherocytes, polychromasia and occasional nucleated RBCs. What is the most likely diagnosis? What additional tests would you do to confirm this diagnosis? • What associated disorders would you be concerned about? • What additional clinical history is important to prevent overtreatment?
Answer 18 • A previously normal person has anemia with spherocytes, polychromasia and occasional nucleated RBCs. • What is the most likely diagnosis? Immune mediated hemolytic anemia • What additional tests would you do to confirm this diagnosis? Coomb’s test • What associated disorders would you be concerned about? Autoimmune, low grade leukemia/lymphoma • What additional clinical history is important to prevent overtreatment? Medication history
66
Heme iron and elemental iron differ in
Their transport into mucosal epithelial cells
67
The PT and APTT are both elevated in
Liver failure Uncompensated DIC
68
A patient with abnormal GP1b complex on the platelets will have an abnormal
Ristocetin aggregation test Platelet aggregation with collagen
69
A patient on heparin for a deep vein thrombosis has a severe drop in platelets 10 days after starting therapy. Two days later he develops ischemic necrosis of his left leg. What caused it?
HITTS, anti-platelet factor 4 heparin complexes activating platelets through FCR2a
70
In severe Von Willebrand disease, there is also an abnormality in which laboratory test?
PTT - b/c lack of factor VIII Platelet aggregation to collagen - VWF mediates adherence to GP1b to collagen Ristocetin - causes aggregation through VWF Ristocetin cofactor
71
A 30 year old patient with a mild sore throat and a newly enlarged nontender, mobile cervical lymph node is most likely to have
Follicular hyperplasia
72
Bcl2 (chr 18)
Follicular lymphoma
73
Cyclin D1 (chr. 11)
Mantle cell lymphoma
74
C-myc (chr 8)
Burkitt lymphoma
75
RAR (chr. 17)
Acute promyelocytic leukemia
76
ZAP-70
CLL/SLL
77
ALK
Anaplastic large cell lymphoma
78
A young child presents with intense bone pain but the X-rays are negative. CBC shows pancytopenia. The patient most likely has
B-ALL is the most common of these choices. There are usually circulating blasts. You should check the smear.
79
When is Tdt expressed?
Tdt is expressed in almost all B-ALL and T-ALL
80
A man presents with an enlarged lymph node with a diffuse pattern of tdt+ B lineage cells. What is this disease called?
Precursor B lymphoblastic lymphoma
81
Can B-ALL and T-ALL be distinguished morphologically
No
82
Most types of B cell lymphomas have translocations to what region? Why? When do they occur?
* **14q32** * **Errors in VDJ recombination in the marrow** * **Errors in somatic hypermutation and class ****switch recombination in the germinal ****center**
83
A patient presents with severe pruritis, splenomegaly and sudden onset of blindness in one eye. Of the following, the patient most likely has
Polycythemia vera Pruritis and thrombosis are problems in PV
84
A patient with an autoimmune hemolytic anemia is found to have 3 g/dL of IgM. He most likely has?
Lymphoplasmacytic lymphoma/ Waldenstrom’s macroglobulinemia
85
A patient from Haiti presents with anemia, neutropenia, thrombocytopenia and lymphocytosis. There are skin lesions and the patient is hypercalcemic, with many lytic bone lesions. Large lymphocytes with clover shaped nuclei are seen in the peripheral blood smear. The patient has
ATLL, associated with HTLV-1
86
A 1 year old boy is presented by his mother because he is always thirsty and has been drinking constantly. Urine test is negative for glucose. Multiple lytic lesions are seen on X-ray. The most likely diagnosis is? What would confirm your diagnosis on immunohistochemistry? On EM?
**Hand-Schuler-Christian syndrome** HSC: lytic skull lesions, diabetes insipidus, exophthalmos Classic triad **(1) Lytic lesion in the skull** * *(2) Diabetes insipidus due to invasion of posterior pituitary (3) Exophthalmos from infiltration of the orbit** **S100** and **CD1a+** cleaved nuclei “histocytic” cells with **Birbeck granules** on EM, admixed with lymphocytes and eosinophils
87
A 20 year old woman presents with a 5 cm cervical lymph node that is fixed. She has no other symptoms. The most likely diagnosis is
Classical is far more common than LP Hodgkin’s
88
50 year old man presents with muscle weakness which is alleviated by an acetylcholinesterase inhibitor. There is a mass in the mediastinum. Histological examination will show
A tumor of epithelial cells with admixed immature T cells OR follicular hyperplasia
89
A patient with splenomegaly and neutropenia has circulating large granular lymphocytes. This disease is
Sometimes associated with autoimmune disorders
90
Colonoscopy performed in a 50 year old man with iron deficiencyanemia shows multiple lymphoid polyps. These are composed ofmonotypic kappa+ B cells. Further examination reveals splenomegaly and lymphadenopathy. The most likely diagnosis is
Frequent GI involvement in Mantle cell lymphoma. Marginal zone B cell lymphoma of MALT type would be very unlikely to involve the spleen and lymph nodes.
91
A patient presents with splenomegaly and anemia. The marrow is inaspirable, and the biopsy shows a fibrotic marrow with an infiltrate of medium sized B cells with a “fried egg” appearance. What else might you expect to see?
Circulating cells with cytoplasmic protrusions - This is a typical presentation of hairy cell leukemia
92
``` A 40 year old man is diagnosed with AML with a t(8;21). This is an example of ```
Good prognosis AML
93
A patient presents with a precursor B lymphoblastic lymphoma in an axillary lymph node. FISH studies reveal a t(9;22) in the lymphoblasts. Peripheral blood shows marked leukocytosis (70,000/mcL) with marked left shift, 5% basophils, and 25% blasts. All of the cells have a Philadelphia chromosome on karyotype, and some show loss of 17p.This is an example of
CML presenting in blast crisis 25% of the time the blasts are lymphoblasts in blast crisis
94
An HIV+ patient presents with a rapidly enlarging neck mass. Biopsy shows a diffuse starry sky proliferation of cells of germinal center origin and cytogenetics show a c-myc translocation. 100% of cells are Ki-67+. Bcl2 is negative. This is an example of
Burkitt’s lymphoma
95
A patient with a longstanding skin rash develops lymphadenopathy. The peripheral blood shows lymphocytosis with medium sized mature-looking cells with convoluted nuclei. Flow cytometry will show that the cells are.. In the previous example, a skin biopsy would show an infiltrate of T cells in a
T cells - Sezary syndrome Upper dermis and epidermis
96
A patient has a Hb of 12.1 with an MCV of 65fL. Hemoglobin electrophoresis is normal. Which of the following is a possible underlying genetic finding?
Deletion of two alpha globin genes
97
A 10 year old boy with a mediastinal mass has a biopsy showing sheets of blasts that express T cell markers by flow cytometry. Tdt is also positive. Which of the following is a cell surface molecule likely expressed on the malignant cells that might be targeted for therapy with a small molecule selective protease inhibitor.
Notch
98
en minutes after sending blood to the laboratory the results of most of the cell counts are available but the platelet count is missing. Which is a possible explanation
A manual smear is being checked for clumps
99
* Dark urine * Moderate anemia * Retic increased * Jaundice * Total bilirubin elevaed * Haptoglobin decreased * Hemosiderine in Urine cytoplasm
Intra vascular hemolysis
100
A patient presents with acute liver failure. Imaging studies show a large clot in the hepatic vein.Routine coagulation studies are normal. The CBC shows pancytopenia with a microcytic hypochrom ic anemia. Which of the following additional tests might establish the correct diagnosis.
CD55/CD59 measurement on the patient ’s blood cells
101
A bone marrow biopsy from 30 year old man with severe pancytopenia shows 90% fat,decreased hematopoieticelements and no leukemic infiltrate. The underlying pathologyis most likely related to
* **Idiopathic Approximately 50-70% of cases are idiopathic** * **Drugs Most common known cause of aplastic anemia** * Dose-related causes are usually reversible (e.g., alkylating agents) * Idiosyncratic reactions are frequently irreversible (e.g., chloramphenicol) * Chemical agents Toxic chemicals in industry and agriculture (e.g., benzene, insecticides-DDT, parathion) * Infection May involve all hematopoietic cell lines (pancytopenia) or erythroid cell line alone (pure RBC aplasia) * Examples-EBV; CMV; parvovirus; non-A, non-B hepatitis, HCV * Physical agents Whole-body ionizing radiation (therapeutic or nuclear accident) * Miscellaneous Thymoma (may be associated with pure RBC aplasia) * Paroxysmal nocturnal hemoglobinuria
102
A six year old boy is diagnosed with precursor B lymphoblastic leukemia. Which ofthe following results wouldnecessitate treatment more aggres sive than for standard risk ALL?
Presence of a chromosomal rearrangement involving the MLL gene
103
The cells have surface immunoglobulin, B cell markers and also express CD5 andCD23. The karyotype shows deletion of 11q23. The diagnosis is
Chronic lymphocytic leukemia with a poor prognosis.
104
The bone marrow is packed with abnormal monocytes and monoblasts with very little normal hematopoiesis. FISH for which of the following loci is likely to show a breakpoint?
MLL
105
Flow cytometric studies show CD20+ CD10+ cells that all express surface kappa immunoglobulin light chain. A t(14;18) is seen on the karyotype
Follicular lymphoma
106
A 53 year old man presents with anemia. Bone marrow examination shows an extensive infiltrate by light chain isotype - restricted mature B cells, plasma cells and intermediate forms. The patient may
Suffer a stroke
107
Primary amyloidosis
Systemic deposition of AL amyloid, derived from immunoglobulin light chain (kappa (c2) or lambda(c22))
108
Type of cells seen in MALT lymphoma?
destructive mucosal infiltrate of medium sized mature B cells
109
Sections show a diffuse infiltrate of medium sized B cells that express CD5
Mantle cell lymphoma
110
lymphocyte predominant Hodgkin’s lymphoma. However, PCR analysis failed to reveal a clonal immunoglobulin heavy chain (IgH) product. This is because
The malignant cells have ongoing IgH gene somatic hypermutation.
111
A 50 year old man with fevers and night sweats is found to have classical Hodgkin’s lymphoma involving cervical nodes and mediastinum. This is Ann Arbor stage
**II A** ## Footnote **A** - absence of B symptoms **B** - B-symptoms (Fever, night sweats, weight loss exceeding 10% of body weight in previous 6 months) **E** for extralymphatic organ site **S** for spleen **Stage I** — Involvement of a single lymph node region (I) or of a single extralymphatic organ or site (IE) **Stage II** — Involvement of two or more lymph node regions on the same side of the diaphragm **Stage III** — Involvement of lymph node regions or lymphoid structures on both sides of the diaphragm (III) which may include the spleen (IIIS) or limited, a contiguous extralymphatic organ or site (IIIE) or both (IIIES)
112
Bone marrow e xamination of a 30 year old man with pancytopenia shows a mixture of blasts and monocyte like cells. The patient was treated three years prior for a testicular tumor. Cytogenetics showed an MLL translocation. Which of the following agents in his prior treatment most likely contributed to his current leukemia.
A topoisomerase II inhibitor Etoposide - ↑ DNA degradation used for Small cell carcinoma of lung, prostate, testicular carcinoma.
113
A patient diagnosed with CML chronic phase initiates treatment with imatinib. After 2 months there is no change in the peripheral blood. This indicates that
There may be a **POINT MUTATION** in the BCR/ABL kinase
114
A patient is found to have a 5 cm mass in the spleen. Which of the following is the most likely malignancy?
**Diffuse large B cell lymphoma** 50% of adults with NHL; elderly and childhood populations Clinically aggressive! Localized disease with extranodal involvement: GI tract, brain (EBV association with AIDS
115
A child with multiple bony sites involved by Langerhans cell histiocytosis is most likely to have which of the following?
Diabetes insipidus
116
ANAPLASTIC LARGE CELL LYMPHOMA
* Always express **CD30** (target of Ki-1 antibody) but not CD15 (distinguishing it from classical Hodgkin’s lymphoma). * **○ ALK+** ▪ t(2;5) NPM/ALK translocation in younger patients, with relatively good prognosis after chemotherapy. **○ ALK-** ▪ Poor prognosis, older patients.
117
most common muscle disease over age 50 years
Inclusion Body Myositis (IBM)
118
associated with acute renal failure.
Indomethacin and Ketorolac
119
Most common treatable neuropathy in the world!
Leprosy Causes a symmetric polyneuropathy involving pain fibers and causing a loss of sensation. ▪ Schwann cells invasion results in segmental demyelination, remyelination, and eventual loss of axons. Tuberculoid - mononeuropathy multiplex (more localized nerve involvement
120
Chronic Demyelinating Polyneuritis
Resembles the Guillain-Barré syndrome, but has a less acute presentation and recovery, and **frequently recurs.** With repeated attacks and remissions, nerve biopsy may show** “onion-bulb” changes**
121
Most common hereditary peripheral neuropathy
Hereditary motor and sensory neuropathy I (Charcot-Marie-Tooth disease, hypertrophic form or Peroneal Muscular Atrophy) ▪ Nerve biopsy shows **segmental demyelination** and remyelination with Schwann cell proliferation and collagen deposition (may find **“onion-bulbs”**, and nerves may be palpably enlarged). ▪ Autopsy study shows degeneration of the posterior columns. ○ Pathogenesis/Genetics (HMSN I) ▪ Genetically heterogeneous (linked
122
DIABETIC NEUROPATHIES
**distal** **predominantly an axonal neuropathy** **Focal, multifocal (cranial nerve lesions, focal lesions of limb nerves), and asymmetric lower limb motor neuropathy(diabetic amyotrophy).**

DHM Block (2 decks)

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