12 - WBCs, lymph nodes, spleen, and thymus Flashcards

1
Q

Most common cause of Agranulocytosis:

A

drug toxicity

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2
Q

Most common cancer in children:

A

ALL

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3
Q

More common type of ALL:

A

B cell ALL

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4
Q

Type of AML with the best prognosis:

A

Acute promyelocytic leukemia

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5
Q

Most common leukemia of adults in the western world:

A

Chronic Lymphocytic leukemia

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6
Q

Most common subtype of Hodgkin lymphoma:

A

nodular sclerosis

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7
Q

All subtypes of Hodgkin lymphoma are more common in males, except:

A

nodular sclerosis (F=M)

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8
Q

All patients with Hodgkin lymphoma in adults are usually adults, except in this subtype:

A

lymphocyte predominance (young males)

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9
Q

Most common indolent lymphoma of adults:

A

follicular lymphoma

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10
Q

Most common form of non-Hodgkin lymphoma:

A

Diffuse large B-cell lymphoma

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11
Q

Most common lymphoma of adults:

A

Diffuse large B-cell lymphoma

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12
Q

Fastest growing human tumor:

A

Burkitt lymphoma

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13
Q

Most important plasma cell neoplasm:

A

multiple myeloma

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14
Q

Decreased number of circulating leukocytes; most commonly neutrophils (neutropenia); deficiency of lymphocytes (lymphopenia) is less common, and is commonly seen in advanced HIV and other diseases.

A

Leukopenia

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15
Q

Clinically significant neutropenia (<500/mm3); highly susceptible to infections (Candida and Aspergillus); most common cause: drug toxicity

A

Agranulocytosis

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16
Q

An increase in the number of white cells in the blood in a variety of inflammatory states caused by microbial and nonmicrobial stimuli that may mimic leukemia.

A

Reactive leukocytosis, Leukemoid reaction (high leukocyte alkaline phosphatase, a product of normal WBCs; used to differentiate it from leukemias)

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17
Q

Enlargement of a lymph node as immune response to foreign antigens; histology usually nonspecific; depends on duration of disease and type of offending agent.

A

Reactive lymphadenitis

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18
Q

Most common type of cancer in children; highly aggressive tumors manifesting with signs and symptoms of bone marrow failure, marrow expansion, dissemination of leukemic cells, and CNS manifestations; lymphoblasts with irregular nuclear contours, condensed chromatin, small nucleoli and scant agranular cytoplasm on BMA; blasts compose >25% of marrow cellularity; TdT(+) in 95% of cases; most responsive to chemotherapy (Asparaginase).

A

Acute lymphoblastic leukemia (ALL)

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19
Q

Clinical differences between B- and T-cell ALL.

A
  • B-cell ALL typically occurs in younger children presenting with BM failure
  • T-cell ALL typically occurs in adolescent males presenting with thymic masses
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20
Q

Good prognostic factors in ALL.

A

Children 2-10 years old; t(12;21) and hyperdiploidy

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21
Q

Poor prognostic factors in ALL.

A
  • Male gender;
  • age younger than 2 or older than 10 years;
  • a high leukocyte count at diagnosis;
  • molecular evidence of persistent disease on day 28 of treatment, t(9;22) and MLL rearrangements
22
Q
  • Most common leukemia of adults in Western world; chronic leukemia associated with BCL2, an antiapoptotic molecule; patient presents with increased susceptibility to infections due to hypogammaglobulinemia; CBC showed >5000 lymphocytes/mm3; histologically, foci of mitotically active cells (proliferation centers) are present; also, smudge cells (due to fragility of circulating tumor cells) are also evident;
  • Syndrome in which this type of leukemia transforms into DLBCL
A
  • Chronic lymphocytic leukemia/Small lymphocytic lymphoma (CLL/SLL)
  • Richter syndrome

SLL: <5000 lymphocytes/mm3

23
Q

Most common indolent lymphoma of adults; frequent small “cleaved” cells mixed with large cells, growth pattern is nodular, centroblasts present; occurs in older adults, usually involves nodes, marrow, spleen; associated with t(14;18) that results in overexpression of cyclin D1.

A

Follicular lymphoma

24
Q

Most common lymphoma of adults; most common form of NHL; tumor cells have large nuclei with open chromatin and prominent nucleoli; most important type of lymphoma in adults, accounting to ~50% of adult NHLs.

A

Diffuse Large B-cell lymphoma (DLBCL)

25
17/M presented with a short history of fever, tonsillitis and unilateral enlarged cervical lymph nodes. PE revealed enlargement of right cervical lymph node, 3 cm in diameter, hard, and pharyngeal hyperemia. Histologically, there was intermediate-sized round lymphoid cells with 2-5 prominent nucleoli. High rates of proliferation and apoptosis are characteristic. Nuclear remnants phagocytosed by interspersed macrophages with abundant clear cytoplasm, "starry sky pattern". Also, it is associated with cMYC oncogene [t(8;14)]. The most likely diagnosis is? Clue: it is also the fastest growing human tumor.
Burkitt lymphoma
26
Disease that presents as multifocal destructive bone lesions seen as punched-out defects on imaging. Renal involvement is also prominent, causing production of proteinaceous casts in the DCT and collecting ducts (that can cause renal insufficiency); can also present with immunodeficiency due to impaired normal plasma cell function.
Multiple myeloma
27
Most common M protein in myeloma cells.
IgG
28
Excess light or heavy chains along with complete Igs synthesized by neoplastic plasma cells.
Bence-Jones protein
29
Basically, CLL/SLL with plasmacytic differentiation; usually present with hyperviscosity syndrome (Waldenstrom macroglobulinemia (IgM)): visual changes, neurologic problems, bleeding diathesis and cryoglobulinemia; no bone manifestations; renal manifestations rare.
Lymphoplasmacytic lymphoma
30
Lymphomas of memory B-cell origin; usually arises on tissues on chronic inflammation; regresses when inciting agent is removed, but once it progresses to DLBCL, regression may not be possible; examples: salivary glands: Sjogren syndrome, thyroid gland: Hashimoto thyroiditis, stomach: H. pylori gastritis
Marginal zone lymphoma
31
These tumors of neoplastic CD4+ T cells home to the skin; usually manifests as a nonspecific erythrodermic rash that progresses over time to a plaque phase and then to a tumor phase; histologically, neoplastic T cells, often with a cerebriform appearance produced by marked infolding of the nuclear membranes, infiltrate the epidermis and upper dermis.
Mycosis fungoides (Cutaneous T-cell lymphoma)
32
Diagnostic cells in HL; large, multiple nuclei or single with multiple lobes; each with nucleolus about a size of a small lymphocyte, CD15(+), CD30(+).
Reed-Sternberg (RS) cells
33
Classical HL types; CD15 and CD30(+).
NSHL, MCHL, LRHL, LDHL
34
Most common variant of HL; with lacunar Reed-Sternberg (RS) cells; with deposition of collage bands that divide lymph node into nodules; not associated with EBV; excellent prognosis; usually diagnosed at early stage (Stage I/II) with frequent mediastinal involvement.
Nodular sclerosis HL (NSHL)
35
Mononuclear and diagnostic RS cells with heterogenous cellular infiltrate; associated with EBV in 70% of cases; \>50% of cases diagnosed at Stage III/IV .
Mixed cellularity HL (MCHL)
36
Mononuclear and diagnostic RS cells with lymphocytic infiltrate; not associated with EBV; excellent prognosis.
Lymphocyte-rich HL (LRHL)
37
Lymphocytes are scarce with relative abundance of diagnostic RS cells; associated with EBV in 90% of cases; associated with PLHIV; worst prognosis.
Lymphocyte-depleted HL (LDHL)
38
Lymphohistiocytic "popcorn" RS cells with nodular infiltrate of small lymphocytes admixed with macrophages; excellent prognosis; CD15 and 30 (-), CD20(+).
Lymphocyte-predominant (LPHL)
39
Staging system used for HL.
Ann-Arbor classification
40
BMA shows hypercellular marrow packed with \_20% myeloblasts (and azurophilic needle-like material called Auer rods (faggot cells)); clinically presents with pancytopenia and bleeding; poor prognosis because it is difficult to treat.
Acute myeloid leukemia (AML)
41
Stains used to differentiate Myeloblasts from Monoblasts.
* Myeloblasts: Myeloperoxidase (MPO)(+) Non-specific esterase (NSE)(-) * Monoblasts: MPO(-), NSE(+)
42
Stains used to differentiate Myeloblasts from lymphoblasts.
Lymphoblasts: MPO(-) PAS(+)
43
Main differences between AML and ALL.
* AML occurs in adults, CNS spread is rare, and is more difficult to treat; * ALL occurs in children, CNS spread is common and is generally responsive to chemotherapy
44
An AML type that usually presents with DIC; associated with t(15;17) translocations; highly responsive to all-trans retinoic acid.
Acute promyelocytic leukemia
45
Disorder of defective hematopoietic maturation that results in ineffective hematopoiesis (cytopenias); more common in the elderly; clinically present as bone marrow failure; associated with increased risk of transformation to AML; poor prognosis.
Myelodysplastic syndrome
46
Common features of chronic myeloproliferative disorders.
Increased proliferative drive in BM, extramedullary hematopoiesis, spent phase, and variable transformation to AML
47
Chronic leukemia associated with BCR-ABL fusion gene (Philadelphia chromosome, t(9;22)); clinically presents with nonspecific symptoms and splenomegaly; BMA shows hypercellular marrow packed with less than 10% myeloblasts with more mature forms; CBC shows leukocytosis \>100,000/mm3 with low leukocyte alkaline phosphatase; and scattered macrophages with abundant, wrinkled, green-blue cytoplasm (sea-blue histiocytes); can proceed to a blast crisis if neglected.
Chronic myelogenous leukemia (CML)
48
Increase in all cell lines, but erythroid lines are more increased; associated with JAK2 mutations in most cases; 2% chance of transformation to AML.
Polycythemia vera (PV)
49
Increase in megakaryotic lines; associated with JAK2 mutations in 50% of case; transformation to AML is uncommon.
Essential thrombocytosis
50
Extensive deposition of collagen in marrow by non-neoplastic fibroblasts; associated with JAK2 mutations in 50-60% of cases; 5-20% chance of transformation to AML.
Primary myelofibrosis