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Jaspie - Pathology > 12 - WBCs, lymph nodes, spleen, and thymus > Flashcards

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
Q

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.

A

Burkitt lymphoma

26
Q

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.

A

Multiple myeloma

27
Q

Most common M protein in myeloma cells.

A

IgG

28
Q

Excess light or heavy chains along with complete Igs synthesized by neoplastic plasma cells.

A

Bence-Jones protein

29
Q

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.

A

Lymphoplasmacytic lymphoma

30
Q

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

A

Marginal zone lymphoma

31
Q

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.

A

Mycosis fungoides (Cutaneous T-cell lymphoma)

32
Q

Diagnostic cells in HL; large, multiple nuclei or single with multiple lobes; each with nucleolus about a size of a small lymphocyte, CD15(+), CD30(+).

A

Reed-Sternberg (RS) cells

33
Q

Classical HL types; CD15 and CD30(+).

A

NSHL, MCHL, LRHL, LDHL

34
Q

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.

A

Nodular sclerosis HL (NSHL)

35
Q

Mononuclear and diagnostic RS cells with heterogenous cellular infiltrate; associated with EBV in 70% of cases; >50% of cases diagnosed at Stage III/IV .

A

Mixed cellularity HL (MCHL)

36
Q

Mononuclear and diagnostic RS cells with lymphocytic infiltrate; not associated with EBV; excellent prognosis.

A

Lymphocyte-rich HL (LRHL)

37
Q

Lymphocytes are scarce with relative abundance of diagnostic RS cells; associated with EBV in 90% of cases; associated with PLHIV; worst prognosis.

A

Lymphocyte-depleted HL (LDHL)

38
Q

Lymphohistiocytic “popcorn” RS cells with nodular infiltrate of small lymphocytes admixed with macrophages; excellent prognosis; CD15 and 30 (-), CD20(+).

A

Lymphocyte-predominant (LPHL)

39
Q

Staging system used for HL.

A

Ann-Arbor classification

40
Q

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.

A

Acute myeloid leukemia (AML)

41
Q

Stains used to differentiate Myeloblasts from Monoblasts.

A
  • Myeloblasts: Myeloperoxidase (MPO)(+) Non-specific esterase (NSE)(-)
  • Monoblasts: MPO(-), NSE(+)
42
Q

Stains used to differentiate Myeloblasts from lymphoblasts.

A

Lymphoblasts: MPO(-) PAS(+)

43
Q

Main differences between AML and ALL.

A
  • 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
Q

An AML type that usually presents with DIC; associated with t(15;17) translocations; highly responsive to all-trans retinoic acid.

A

Acute promyelocytic leukemia

45
Q

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.

A

Myelodysplastic syndrome

46
Q

Common features of chronic myeloproliferative disorders.

A

Increased proliferative drive in BM, extramedullary hematopoiesis, spent phase, and variable transformation to AML

47
Q

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.

A

Chronic myelogenous leukemia (CML)

48
Q

Increase in all cell lines, but erythroid lines are more increased; associated with JAK2 mutations in most cases; 2% chance of transformation to AML.

A

Polycythemia vera (PV)

49
Q

Increase in megakaryotic lines; associated with JAK2 mutations in 50% of case; transformation to AML is uncommon.

A

Essential thrombocytosis

50
Q

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.

A

Primary myelofibrosis

Jaspie - Pathology (29 decks)

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