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Heme Lymph Exam II > Leukemia, Lymphoma, & White Cell Disorders > Flashcards

Leukemia, Lymphoma, & White Cell Disorders Flashcards

1
Q

> 20% blasts found on bone marrow aspirate/biopsy confirms the diagnosis of _____

A

AML

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2
Q

In AML, myeloblasts express stem cell marker _____, as well as HLA-DR, and markers of granulocyte maturation such as ____ and _____

A

CD34; CD33, CD13

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3
Q

What translocation is associated with the diagnosis of AML with maturation, and is the most frequent abnormality in kids with AML?

A

t(8;21)

Involves RUNX-AML1 gene which encodes core-binding factor required for hematopoiesis

May be accompanied by loss of a sex chromosome, or a del(9q) with loss of 9q22

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4
Q

T/F: t(8;21) is associated with a favorable px in adults, but a poorer px in children

A

True

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5
Q

_____ and _____ are cytogenetic changes associated with Acute Myelomonocytic Leukemia (AMML)

A

Inv(16) and t(16;16)

[breakpoint occurs in CBFB gene and affects RUNX1/AML1 pathway regulating hematopoeisis]

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6
Q

Leukemia characterized by abnormal eosinophils including large irregular basophilic granules and positive reactions with PAS and chloroacetate esterase

A

AMML

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7
Q

AMML with inv(16) and t(16;16) is associated with a good px with intensive chemotherapy, but prognosis is worse if _____ mutation is also present

A

KIT

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8
Q

Translocation associated with acute promyelocytic leukemia

A

t(15;17)

Involves disturbance of retinoic acid receptor resulting in PML-RARA fusion —> RA repression via HDAC-dependent chromatin remodeling

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9
Q

APL with t(15;17) translocation is sensitive to therapy with ______________

Mutations that cooperate with PML-RARA are _____ internal tandem duplications (ITDs)

A

All-trans retinoic acid

FLT3

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10
Q

FLT3-ITD mutations may occur in any AML subtype but most commonly in ____ and AML with a normal karyotype. These mutations are associated with a _____ prognosis

A

APL; poor

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11
Q

NPM1 mutations most commonly involve exon 12 leading to alterations at C-terminus, replacement of tryptophan at position 288 and 290 causing the protein to be aberrantly localized to the cytoplasm. What do NPM1 mutations in AML tell you about the prognosis?

A

Associated with a favorable px in the absence of FLT3 mutations

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12
Q

2 major risk factors for development of AML

A

History of radiation or benzene exposure

History of chemotherapy with alkylating agents

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13
Q

Condition in which leukemic blasts occlude microcirculation leading to complications such as respiratory failure and cerebral dysfunction

A

Leukostasis syndrome

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14
Q

Presentation of AML including locations of extramedullary invasion

A

Nonspecific sxs such as fatigue, pallor from anemia, bleeding issues like bruising and petechiae (d/t underlying thrombocytopenia), increased infections, variable degree of LAD and HSM

Extramedullary invasion of gingivae, skin, and meninges [gingival hypertrophy typically associated w/M4 and M5 variants of AML]

Common complaint is “bone pain”

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15
Q

Clinical/lab findings associated with AML

A

Elevated leukocyte count
Anemia
Thrombocytopenia
Blasts on peripheral smear

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16
Q

3 phases of tx for AML

A

Phase 1 = Induction — 3 days of anthracycline (daunorubicin or idarubicin), 7 days of continuous infusion of cytarabine

Phase 2 = Consolidation — High dose Ara-C (HDAC)

Phase 3 = Maintenance — ATRA (given only if APL subtype)

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17
Q

Major risk associated with induction phase of AML tx

A

Tumor lysis syndrome — characterized by abrupt necrosis of large mass of leukemia cells and release of their contents into circulation; pancytopenia, infections, bleeding complications

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18
Q

t(15;17) translocation, proliferation of promyelocytes with their primary granules

Presents with significant bleeding d/t fibrinolysis and DIC

A

APL

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19
Q

Tx for APL is initiated with all-trans retinoic acid (ATRA), what are the 2 major purposes of this tx?

A

Induces maturation of promyelocyte

Ameliorates DIC

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20
Q

3 phases of APL tx

A
  1. Induction with ATRA + arsenic trioxide
  2. Consolidation with HDAC
  3. Maintenance with ATRA
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21
Q

Most common rearrangement in adult ALL

A

t(9;22) —> BCR-ABL1 fusion (Ph chromosome)

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22
Q

ALL is usually of B cell lineage (CD10, CD19, TdT), but can have myeloid markers including CD13 and CD33. BCR-ABL1 fusion may be associated with _____ gene alterations, which typically indicate ____ px

A

IKZF1; poor

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23
Q

What translocation is associated with ALL in which cells have cytoplasmic Ig, CD10+, CD19+, CD34-, and CD9+

A

t(1;19)

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24
Q

Implications of t(8;14) translocation in ALL

A

Indicates mature B cell ALL — typically associated with high incidence of CNS involvement and/or abdominal nodal involvement at dx

Poor outcome; improved with high intensity chemotherapy

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25
Q

Implications of t(4;11) in ALL

A

Denotes translocation involving KMT2A

Associated with very immature immunophenotype and poor px

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26
Q

Symptoms and PE findings associated with ALL

A

More common in kids, but may occur in adults (usually 7th decade)

Circulating lymphoblasts and progressive marrow failure

Presents w/fatigue, dyspnea, bleeding, and infection-related fever. LAD and HSM are common; CNS involvement may occur

Severe cytopenias and metabolic derangements d/t tumor lysis syndrome are also common

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27
Q

3 tx phases for ALL

A
  1. Induction — anthracycline, cincristine, L-asparaginase, and a glucocorticoid
  2. Consolidation — multiple agents + CNS prophylaxis (MTX or Ara-C)
  3. Maintenance — 6-MP and MTX
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28
Q

What are treatment considerations regarding the risks of CNS involvement and tumor lysis syndrome in ALL?

A

Risk of CNS involvement often requires intrathecal chemoprophylaxis

Risk of tumor lysis syndrome requires IV fluid hydration and allopurinol

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29
Q

Significance of t(9;22) in CML

A

BCR-ABL fusion — results in truncated Chr 22 (Philadelphia Chromosome)

Leads to production of a unique tyrosine kinase protein

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30
Q

Common symptoms and clinical findings with CML

A

Presents w/ fatigue, lethargy, low-grade fever, and weight loss

Splenomegaly may be striking and associated with early satiety, abdominal distention, or LUQ pain

PE may reveal pallor and splenomegaly (LAD uncommon)

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31
Q

Phases in disease course of CML

A

Chronic phase = pts usually present in this phase; may do well for years w/o treatment

Accelerated (blast) phase = characterized by progressive leukocytosis and splenomegaly, extreme thrombocytosis or thrombocytopenia, and systemic symptoms resistant to treatment

Transformation phase = CML typically transforms into AML, but may also transform into ALL

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32
Q

2 main treatment options for CML

A

Imatinib = inhibition of Ph chromosome tyrosine kinase —> reduced leukocyte count, shrinks spleen, and clears bone marrow of Ph chromosome-positive cells [newer agents include dasatinib and nilotinib]

Most definitive cure for CML is allogeneic BMT — but risks are high including mortality d/t graft vs. host disease

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33
Q

How long is the course of imatinib tx for CML?

A

Imatinib must be given indefinitely

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34
Q

Significance of trisomy 12, hypogammaglobulinemia, and autoimmune antibodies in CLL

A

Trisomy 12 = component of CML genotype

Hypogammaglobulinemia = immune defect predisposing to infectious complications, most commonly with encapsulated organisms (e.g., streptococcus pneumoniae)

Autoimmune antibodies = increased risk for autoimmune disease, most commonly autoimmune thrombocytopenia

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35
Q

What is Richter syndrome?

A

Transformation of CLL to DLBCL

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36
Q

Significance of CD38 and ZAP-70 in CLL

A

CD38 is expressed in B-CLL; likely denotes worse px

ZAP-70 expression is also associated with worse px

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37
Q

Rai staging system and prognostic implications for CLL

A

Stage 0 = asymptomatic with lymphocytosis (ALC > 10,000); survival >10 years (150 mos)

Stage 1 = Lymphocytosis + LAD; survival 6-9 yrs (101 mos)

Stage 2 = Lymphocytosis + LAD + HSM; survival 6-9 years (71 mos)

Stage 3 = Lymphocytosis, LAD, HSM, anemia (Hb < 11); survival <2 yrs (22 mos)

Stage 4 = Lymphocytosis, LAD, HSM, anemia, thrombocytopenia; survival <2 years (11 mos)

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38
Q

Treatment goals for CLL include slowing the rate of progression, inducing a period of remission, and controlling symptoms/complications. Young pts may be considered for HSC transplant. What are the typical tx options offered for CLL?

A

Purine analogs — fludarabine, pentostatin

Alkylating agents — chlorambucil, bendamustine, cyclophosphamide

Monoclonal Abs against CD20 — rituximab, ofatumumab

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39
Q

What is the significance of TRAP stain, CD11c, soluble CD25, and BRAF V600E in hairy cell leukemia?

A

TRAP stain = tests for tartrate-resistant acid phosphatase expressed by tumor cells

CD11c = part of HCL immunophenotype

Soluble CD25 = excellent tumor marker for monitoring disease activity

BRAF V600E = activation mutation found in most cases

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40
Q

HCL commonly presents with ______ and pancytopenia. Bone marrow aspirate may reveal a _______ due to increased reticulin and bone marrow fibrosis. Monocytopenia leads to a predisposition for ______ infections

A

Splenomegaly; dry-tap; mycobacterial

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41
Q

General tx approaches for HCL

A

Splenectomy may prolong remission

Nucleosides (cladribine, deoxycoformycin) are highly active but associated with immunosuppression

IFN-alpha also works but less effective than nucleosides

Chemotherapy-refractory pts may respond to vemurafenib

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42
Q

Hodgkin lymphoma and non-hodgkin lymphoma can only be definitively diagnosed by what method?

A

Tissue biopsy

[typically excisional bx is preferred so that pathologist can evaluate architecture AND morphology]

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43
Q

Mutations in the _____ oncogene (most commonly t(8;14)) is associated with highly aggressive Burkitt lymphoma

A

c-MYC

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44
Q

Elevated LDH raises suspicion for _____

A

NHL

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45
Q

All pts suspected of having NHL should be evaluated with a tissue biopsy as well as what other tests?

A

Immunohistochemical staining and flow cytometry

CBC, CMP, serum calcium, serum uric acid, LDH, HIV test, Hep B and C test, PET/CT scan

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46
Q

Hodgkins lymphomas are all of B cell origin. Describe further classification of hodgkins lymphoma

A
  1. Classical HL = most common form — subtypes of classical HL include nodular sclerosis (most common subtype), mixed cellularity, lymphocyte rich, and lymphocyte depleted
  2. Nodular lymphocyte predominant HL (NLPHL)
47
Q

Presentation of Hodgkin’s lymphoma

A

Painless peripheral LAD, usually in cervical and/or supraclavicular region

Second most common presentation is mediastinal mass on CXR

Up to half of pts experience “B symptoms” as well as generalized pruritis

48
Q

Up to 1/2 of HL pts experience B symptoms — what are B symptoms?

A

Body temp >100.4 (38 C)

Weight loss of >10%

Drenching night sweats

49
Q

NHL is most commonly of B cell origin (80-85%) but may also be of T cell or NK cell origin. It may be categorized as indolent, aggressive, or highly aggressive. What is the difference in presentation between these categorizations?

A

Indolent — often present with slowly growing LAD, enlarged spleen or liver, or abnormalities on CBC (anemia, thrombocytopenia, leukopenia)

Aggressive or highly aggressive — present more acutely with rapidly enlarging LNs or rapidly growing mass; more likely to have B symptoms, elevated LDH, and elevated uric acid

50
Q

Age distribution, viral association, and other demographic features of HL

A

Bimodal age distribution — initial peak age 15-34, second peak age 55-70

Suggested association with EBV (i.e., pts with hx of infectious mono)

Also seen in higher frequency in HIV/AIDs pts

NLPHL typically affects men age 30-50 and behaves in indolent fashion

51
Q

Age distribution and other demographic associations with NHL

A

Incidence generally increases with age

Known association with autoimmune disease and/or immunosuppression (HIV/AIDs, organ transplant)

Some types associated with infectious agents — EBV, H.pylori, Chlamydia psitacci, Hep C

52
Q

Basic Ann Arbor staging system for lymphoma

A

Stage I = involvement of single LN region (I) or single extranodal organ or site (IE)

Stage II = involvement of 2+ LN regions or lymphatic structures on the same side of the diaphragm (II) or with involvement of limited, contiguous extranodal tissue (IIE)

Stage III = involvement of LN regions or lymphoid structures on both sides of the diaphragm (III); may involve spleen (IIIS) or limited contiguous extranodal tissue (IIIE)

Stage IV = diffuse or disseminated involvement of 1+ extranodal organs or tissues, with or without lymphatic involvement

53
Q

Stage modifiers utilized in Ann Arbor staging system for lymphoma

A

A = absence of B symptoms

B = presence of B symptoms

E = extranodal site or organ

X = bulky disease (more than 10 cm)

54
Q

Increased levels of beta-2-microglobulin are ______ prognostic indicators for HL and NHL

A

Poor — higher levels of B2M are associated with higher stages of disease

55
Q

Prognostic risk factors are used to guide tx of choice and length of therapy for HL. Adverse risk factors vary for disease stage, but what are some common ones?

A

Elevated ESR, male gender, age 40+, stage IV disease, and bulky mediastinal LAD

56
Q

All pts undergoing tx for lymphoma should undergo baseline _____ and _____ function testing

A

Pulmonary; cardiac

57
Q

Most common chemotherapy regimen for HL — including length of tx by disease stage

A

4-drug ABVD = doxorubicin, bleomycin, vinblastine, dacarbazine

Early stage disease usually receives 2-3 months of chemo followed by a course of radiation to involved region

Advanced stage disease usually receives 6 months of chemo alone; radiation may then be offered to select pts as consolidation therapy

58
Q

define and separate indolent, aggressive, and highly aggressive NHL based on survival

A

Indolent = low-grade; median survival 10-15 years — generally considered incurable but pt survival may be long

Aggressive = intermediate-grade; curable with chemoimmunotherapy, cure rates vary w/underlying biology and other prognostic factors

Highly aggressive = high-grade; curable with chemoimmunotherapy, cure rates vary w/underlying biology and other prognostic factors

59
Q

T/F: T cell lymphomas generally have a better px than B cell lymphomas

A

False, T cell lymphomas generally have worse px

60
Q

Indolent NHL may often be observed w/o tx, and nearly always responds to chemoimmunotherapy when it becomes indicated. It is generally considered incurable but pt survival may be long. What tx is used in the case of B cell lymphoid malignancies?

A

Cytotoxic chemotherapy + Rituximab (anti-CD20 monoclonal Ab)

61
Q

Define “leukoerythroblastic”

A

Leukemoid reaction (increase in WBCs, usually d/t inflammatory condition or infection) + Nucleated RBCs

62
Q

Lifestyle/behavioral causes of neutrophilia

A

Smoking

Obesity

63
Q

Common medications that provoke neutrophilia

A

Corticosteroids
Lithium
Exogenous growth factors (e.g., G-CSF)

64
Q

Name the “pools” of neutrophils

A

Circulating pool
Marginated pool

Storage pools = bone marrow and tissues

65
Q

What is neutrophil demargination?

A

Shift of cells from marginal pool to circulating pool

May be caused by vigorous exercise, acute physical or emotional stess, infusion of epinephrine, etc. Dependent on release of neutrophils from the spleen and redistribution from other vascular beds (particularly pulmonary capillaries)

66
Q

T/F: demargination can account for about a doubling of neutrophil count

A

True — thus if neutrophil count more than doubles, especially in the presence of elevated lymphocyte and monocyte counts, other processes including malignancy should be considered

67
Q

One common cause of eosinophilia is parasitic disease. For example, what disease may arise in the setting of ingestion of raw pork and what are the signs/symptoms?

A

Trichinella spiralis — eosinophilia, periorbital edema, myositis, fever

68
Q

What allergic diseases are associated with eosinophilia?

A 
Allergic rhinitis
Atopic dermatitis
Urticaria/angioedema
Fungal allergy
Asthma
69
Q

What medication classes are associated with the potential for causing eosinophilia?

A

Antibiotics
NSAIDs
Antipsychotics

70
Q

Other causes for eosinophilia include:

Bronchiectasis/cardiac failure

_____ _____ — signs include fatigue, hypotension, and hyperpigmentation

______ ______ — signs include rhinosinusitis, asthma, and eosinophilia

A

Adrenal insufficiency

Churg-strauss syndrome (eosinophilic granulomatosis w/polyangiitis)

71
Q

What history questions should be asked when evaluating for causes of eosinophilia?

A

Ask about B symptoms, rash, diarrhea, allergic sxs, travel hx, food intake

72
Q

Chemotherapeutics that predispose to neutropenia

A 
Alkylating agents
Antimetabolites
Anthracyclines (doxorubicin, daunorubicin)
Taxanes (paclitaxel, docetaxel)
Topoisomerase inhibitors (etoposide)
Platinum analogs (cisplatin)
Gemcitabine
Vinorelbine
73
Q

Antibiotics that contribute to neutropenia

A 
Cephalosporins
Clindamycin
Gentamycin
Sulfonamides
Tetracyclines
Vancomycin
74
Q

Anticonvulsants, antidepressants, and H2 blockers that contribute to neutropenia

A

Anticonvulsants: carbamazepine, mephenytoin, phenytoin

Antidepressants: amitryptiline

H2 blockers: ranitidine

75
Q

CV drugs, diuretics, antipsychotics, and gout meds that contribute to neutropenia

A

CV drugs: hydralazine, captopril, propranalol

Diuretics: chlorothiazide, HCTZ

Antipsychotics: chlorpromazine

Gout: allopurinol

76
Q

Define febrile neutropenia as well as its associated signs/symptoms

A

Fever >101 for 1 hr with ANC <500

Associated with recent chemotherapy, tachycardia, hypotension, chills, confusion, dysuria, diarrhea, SOB, sore throat, pain/redness/swelling around infusion device or port where chemo is being given

77
Q

Indications for performing a bone marrow exam

A

Diagnosis, staging, and therapeutic monitoring for lymphoproliferative d/o (CLL, HL, NHL), hairy cell leukemia, myeloproliferative disorders, myelodysplastic syndromes, and multiple myeloma

78
Q

Define bone marrow “dry tap” and 2 common causes

A

Dry tap = inability to aspirate bone marrow

Common causes include hairy cell leukemia and primary myelofibrosis

79
Q

Chronic infections that cause neutrophil sequestration (hypersplenism)

A 
Tuberculosis
Brucellosis
Typhoid fever
Malaria
Kala azar
80
Q

Benzene exposure and previous therapy with alkylating agents are huge risk factors for ____

A

AML

81
Q

Prior radiation exposure provides a risk factor for all leukemias except ____

A

CLL

82
Q

What is sweet’s syndrome?

A

Aka acute febrile neutrophilic dermatosis

Cutaneous manifestation of AML — bx shows myeloblasts in the dermis (histiocytic variant of AFND)

83
Q

Auer rods are azurophilic rods in the cytoplasm of pts with ______

A

AML

84
Q

Multiple smudge cells on histology indicates ____

A

CLL

85
Q

34 y/o male w/ rapid onset of fatigue and easy bruisability. He reports one episode of chills and fever to 101.2. PE shows mild scleral icterus and a red spot is seen on the sclera. He has palatal petechiae and a tender palpable spleen 2 cm below the lower costal margin. These findings suggest a chronic hematologic problem. The pts CMP is significant for a K 5.4, LDH 2750, Bili 1.2, Uric acid 12.9, BUN 37, Creat 1.1. His PT is 22.7 and PTT 70. He is not taking anticoagulants. His labs might also demonstrate:

A. A fibrinogen of 400
B. A d-dimer of <0.4
C. Schistocytes
D. A fibrinogen of 50
E. Increased lysozyme level
A

D. A fibrinogen of 50

86
Q

34 y/o male w/ rapid onset of fatigue and easy bruisability. He reports one episode of chills and fever to 101.2. PE shows mild scleral icterus and a red spot is seen on the sclera. He has palatal petechiae and a tender palpable spleen 2 cm below the lower costal margin. These findings suggest a chronic hematologic problem. The pts CMP is significant for a K 5.4, LDH 2750, Bili 1.2, Uric acid 12.9, BUN 37, Creat 1.1. His PT is 22.7 and PTT 70. He is not taking anticoagulants. This pt most likely has:

A. TTP
B. HCL
C. APL
D. ALL
E. CML
A

C. APL

87
Q

34 y/o male w/ rapid onset of fatigue and easy bruisability. He reports one episode of chills and fever to 101.2. PE shows mild scleral icterus and a red spot is seen on the sclera. He has palatal petechiae and a tender palpable spleen 2 cm below the lower costal margin. These findings suggest a chronic hematologic problem. The pts CMP is significant for a K 5.4, LDH 2750, Bili 1.2, Uric acid 12.9, BUN 37, Creat 1.1. His PT is 22.7 and PTT 70. He is not taking anticoagulants. This pt most likely has APL. What should he be treated with?

A. FFP and platelet transfusion
B. 2-chlorodeoxyadenosine
C. ATRA
D. Vincristine + prednisone
E. Decitabine
A

C. ATRA

[D is tx for ALL; E is used for myelodysplastic syndromes]

88
Q

34 y/o male w/ rapid onset of fatigue and easy bruisability. He reports one episode of chills and fever to 101.2. PE shows mild scleral icterus and a red spot is seen on the sclera. He has palatal petechiae and a tender palpable spleen 2 cm below the lower costal margin. These findings suggest a chronic hematologic problem. The pts CMP is significant for a K 5.4, LDH 2750, Bili 1.2, Uric acid 12.9, BUN 37, Creat 1.1. His PT is 22.7 and PTT 70. He is not taking anticoagulants. The pt most likely has APL. The most likely finding on cytogenetic analysis for this pt is:

A. t(12;21)
B. Trisomy 8
C. t(8;21)
D. 5q-
E. t(15;17)
A

E. t(15;17)

[A is M1 and M2 AML, B is in myelodysplastic syndromes and sometimes AML, C is M1 AML, D is myelodysplasia]

89
Q

An 18 y/o female presents w/increasing fatigue and blurry vision x4 days. Her cervical and supraclavicular nodes are palpable and nontender. The spleen is palpated 3 finger breadths below the LCM. Neuro exam demonstrates mild diplopia that resolves with covering the left eye. Her WBCs are 118K w/10% segs, 18% lymphs, 80% blasts, 2% monos; Hb 9.1/Hct 28.2%; plts 101K. CMP: LDH 1760, Uric A 8.6, BUN 30, Creat 0.9. Her PT 11.8, PTT 22.3. She most likely has

A. AML
B. CLL
C. Anaplastic T-cell lymphoma
D. ALL
E. HCL
A

D. ALL

90
Q

An 18 y/o female presents w/increasing fatigue and blurry vision x4 days. Her cervical and supraclavicular nodes are palpable and nontender. The spleen is palpated 3 finger breadths below the LCM. Neuro exam demonstrates mild diplopia that resolves with covering the left eye. Her WBCs are 118K w/10% segs, 18% lymphs, 80% blasts, 2% monos; Hb 9.1/Hct 28.2%; plts 101K. CMP: LDH 1760, Uric A 8.6, BUN 30, Creat 0.9. Her PT 11.8, PTT 22.3. She most likely has ALL. Her neurologic findings are most likely d/t

A. TIA
B. Acute intracranial hemorrhage
C. Leukostasis
D. Leptomeningeal carcinomatosis
E. Increased intracranial pressure
A

D. Leptomeningeal carcinomatosis

[ALL likes to involve the brain and meninges; most common presenting complaint of ppl who have carcinomatosis of the brain is blurry vision]

91
Q

An 18 y/o female presents w/increasing fatigue and blurry vision x4 days. Her cervical and supraclavicular nodes are palpable and nontender. The spleen is palpated 3 finger breadths below the LCM. Neuro exam demonstrates mild diplopia that resolves with covering the left eye. Her WBCs are 118K w/10% segs, 18% lymphs, 80% blasts, 2% monos; Hb 9.1/Hct 28.2%; plts 101K. CMP: LDH 1760, Uric A 8.6, BUN 30, Creat 0.9. Her PT 11.8, PTT 22.3. She most likely has ALL. Her bone marrow exam will demonstrate what cytogenic abnormality?

A. t(8;21)
B. Trisomy 8
C. t(4;11)
D. 7q-
E. Inv(16)
A

C. t(4;11)

92
Q

An 18 y/o female presents w/increasing fatigue and blurry vision x4 days. Her cervical and supraclavicular nodes are palpable and nontender. The spleen is palpated 3 finger breadths below the LCM. Neuro exam demonstrates mild diplopia that resolves with covering the left eye. Her WBCs are 118K w/10% segs, 18% lymphs, 80% blasts, 2% monos; Hb 9.1/Hct 28.2%; plts 101K. CMP: LDH 1760, Uric A 8.6, BUN 30, Creat 0.9. Her PT 11.8, PTT 22.3. She most likely has ALL with leptomeningeal carcinomatosis. In addition to systemic tx, which therapy will be needed for her diplopia?

A. Radial keratotomy
B. Intrathecal methotrexate
C. High dose cytrabine
D. Corrective lenses
E. Neurosurgical decompression
A

B. Intrathecal methotrexate

93
Q

A 77 y/o man comes in for 6 month check up. He reports no new complaints and is feeling well. His exam is significant for small b/l cervical LAD. His WBC count is 22.5 w/50% segs, 45% lymphs, 4% monos, 1% eos. His Hb is 14.1/Hct 47% and plts 324K. The most likely explanation for the pts findings is

A. A lymphoproliferative d/o
B. Prolymphocytic leukemia
C. Hairy cell leukemia
D. An indolent lymphoma
E. Kikuchi disease
A

A. A lymphoproliferative d/o

[kikuchi disease is benign lymphoproliferative d/o reported in Asians affected with diffuse LAD, fever, etc. with reactive lymphoid rxn on bx]

94
Q

Pain in LNs after beer drinking is associated with _____

A

HL

95
Q

Signs of retroperitoneal LN enlargement

A

Back pain

Leg edema

96
Q

Immunosuppressed pts (HIV, organ transplant recipients, etc.) are at increased risk for _______ _____ lymphoma

A

Primary CNS lymphoma

97
Q

EBV is associated with what 2 lymphomas?

A

HL, Burkitts

98
Q

H.pylori is associated with what lymphoma?

A

Gastric MALT lymphoma

[tx with abx and PPI]

99
Q

A 24 y/o female presents w/ localized soft tissue swelling of superior portion of posterior neck. No hx of fever or chills. The LN has firm, rubbery consistency. Her exam and labs are otherwise normal. The best plan for management at this point is

A. Observation
B. Empiric abx
C. FNA
D. Surgical excision
E. CT of the head/neck
A

D. Surgical excision

100
Q

A 24 y/o female presents w/ localized soft tissue swelling of superior portion of posterior neck. No hx of fever or chills. The LN has firm, rubbery consistency. Her exam and labs are otherwise normal. Bx confirms HL. The next step in management is

A. Staging evaluation
B. Lymphadenectomy
C. Course of radiotherapy
D. Observation
E. ABVD chemo
A

A. Staging evaluation

[then would likely proceed with CT/PET imaging (neck, chest, abdomen, pelvis), and bone marrow exam

101
Q

A 24 y/o female presents w/ localized soft tissue swelling of superior portion of posterior neck. No hx of fever or chills. The LN has firm, rubbery consistency. Her exam and labs are otherwise normal. Bx confirms HL and staging demonstrates stage IA disease. The next step in management is

A. Stem cell transplant
B. Lymphadenectomy
C. Course of radiotherapy
D. Observation
E. ABVD chemo
A

C. Course of radiotherapy

[note: Stage IA = 1 site on one side of diaphragm, A indicates asymptomatic]

102
Q

A 68 y/o F presents with painless swelling in groin x2 months. She has no other complaints and there are not infectious symptoms. Her exam is significant for 2 cm soft rubbery nontender LN in the left femoral region. A neoplastic etiology is suspected. Your leading concern is for

A. Diffuse small non-cleaved (Burkitt) lymphoma
B. Hodgkin lymphoma
C. Indolent lymphoma
D. Melanoma
E. Sarcoidosis
A

C. Indolent lymphoma

[note: diffuse small non-cleaved burkitt lymphoma has one of the most aggressive courses known w/tumor doubling time of just 36 hours]

103
Q

A 68 y/o F presents with painless swelling in groin x2 months. She has no other complaints and there are not infectious symptoms. Her exam is significant for 2 cm soft rubbery nontender LN in the left femoral region. A neoplastic etiology is suspected. Staging eval demonstrates mild para-aortic and retroperitoneal adenopathy without HSM. The best approach to management is

A. Administration of rituximab
B. Stem cell transplant
C. Administration of R-CHOP chemo
D. Observation
E. Involved field radiotherapy
A

D. Observation

104
Q

An 18 y/o woman presents w/increasing fatigue and a new mass in her neck. PE demonstrates a 7 cm mass emanating from left supraclavicular region and spleen tip palpable on deep inspiration. She is listless and has frequent drenching night sweats. CBC is normal, as is her CMP but for LDH 1140 and T.bili 2.1. The next step in management should be

A. Staging exam
B. Excision bx of the mass
C. Panculture
D. Hepatology consult
E. Bone marrow exam
A

B. Excision bx of the mass

105
Q

An 18 y/o woman presents w/increasing fatigue and a new mass in her neck. PE demonstrates a 7 cm mass emanating from left supraclavicular region and spleen tip palpable on deep inspiration. She is listless and has frequent drenching night sweats. CBC is normal, as is her CMP but for LDH 1140 and T.bili 2.1. LN bx shows diffuse infiltrate of atypical lymphoid cells w/numerous mitoses and prominent starry sky pattern. Cytogenetics should demonstrate

A. t(14;18)
B. 7q-
C. t(6;9)
D. t(8;14)
E. t(9;22)
A

D. t(8;14)

106
Q

18 y/o woman presents w/increasing fatigue and a new mass in her neck. Staging exam shows bulky disease in left supraclavicular area, mild mediastinal disease, and splenomegaly. Her ann arbor stage is

A. IA
B. IIA
C. IVA
D. IIIAS
E. IIIBS
A

E. IIIBS

107
Q

18 y/o woman presents w/increasing fatigue and a new mass in her neck. Staging exam shows bulky disease in left supraclavicular area, mild mediastinal disease, and splenomegaly. Her ann arbor stage is IIIBS. The best option for tx is

A. Observation
B. Involved field radiation therapy
C. R CHOP chemo
D. ALL-style chemo regimen
E. Stem cell transplant
A

D. ALL-style chemo regimen

108
Q

A 40 y/o male presents w/chest tightness. PMH includes CAD s/p cardiac cath demonstrating dz in circumflex and 2nd diagonal of 50%. PE shows mild facial swelling and prominence of EJV in neck; the superficial vv. of his chest are mildly distended and his spleen is 5 cm below LCM. CBC shows WBC 12.4 w/75% segs, 2% bands, 22% lymphs, 1% monos, Hb 11.5/Hct 36%; plts 167K. CMP shows LDH 750, Ca 11.0. Biopsy of mediastinum demonstrates a mature lymphoma w/an absence of surface Ig and ALK positivity. BM exam is positive. His ann arbor stage is

A. IA
B. IIB
C. IIIA
D. IIIAS
E. IV
A

E. IV

109
Q

A 40 y/o male presents w/chest tightness. PMH includes CAD s/p cardiac cath demonstrating dz in circumflex and 2nd diagonal of 50%. PE shows mild facial swelling and prominence of EJV in neck; the superficial vv. of his chest are mildly distended and his spleen is 5 cm below LCM. CBC shows WBC 12.4 w/75% segs, 2% bands, 22% lymphs, 1% monos, Hb 11.5/Hct 36%; plts 167K. CMP shows LDH 750, Ca 11.0. Biopsy of mediastinum demonstrates a mature lymphoma w/an absence of surface Ig and ALK positivity. BM exam is positive. Ann Arbor stage IV. The best approach to tx is

A. Observation
B. R-CHOP chemo
C. CHOP chemo
D. CHOP chem followed by radiotherapy to residual disease
E. Stem cell transplant
A

D. CHOP chem followed by radiotherapy to residual disease

110
Q

Gene coding for neutrophil elastase, seen in cyclic neutropenia

A

ELANE

111
Q

20 y/o female w/hx of anaphylactic allergy to nuts unknowingly consumes salad containing walnuts. She self injects her epi pen then presents to the clinical for evaluation. CBC shows WBC 22.6 w/normal differential. She has mild wheezing and appears anxious with a dry cough. WBC is most likely elevated due to

A. Aspiration PNA
B. Anxiety
C. Epinephrine admin
D. Acute phase reaction
E. Academic stress
A

C. Epinephrine admin

112
Q

74 y/o woman presents w/diffuse arthralgia and myalgia which began 3 days ago. One week ago, she was placed on oral ciprofloxacin 750mg TID for a UTI. She has no fever or chills, and has no prior hx of these complaints. Her urinalysis demonstrates no bacteria, but 20-30 WBC/hpf are noted in her urine eval. CBC demonstrates total WBC 9800 with 30% eosinophils. ESR is 40mm/hr. Her complaints are most likely representative of

A. Eosinophilia-myalgia syndrome
B. Rheumatoid arthritis
C. Urosepsis
D. Fungemia
E. Parasitic infection
A

A. Eosinophilia-myalgia syndrome

[MCC of this is fluoroquinolones like ciprofloxacin]

113
Q

triad of felty syndrome

A

Rheumatoid arthritis

Splenomegaly

Neutropenia

Heme Lymph Exam II (13 decks)

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