Pathology of White Blood Cells, Lymph Nodes, Spleen, and Thymus (Part 3 of 4) Flashcards

1
Q

What are the three types of plasma cell neoplasia?

A

multiple/plasma cell myeloma, monoclonal gammopathy of uncertain significance, and Waldenstrom macroglobulinemia

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2
Q

What are 4 features that are consistent with myeloma? (4)

A

lytic bone lesions, hypercalcemia, renal failure, immune abnormalities

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3
Q

What is myeloma?

A

the primary tumor of bone marrow

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4
Q

Histologically speaking, what is the predominant feature of myelomas?

A

plasma cells- but these are clones

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5
Q

What stains can you use to diagnose myelomas?

A

CD138, Kappa, and Lambda

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6
Q

What can a monoclonal gammopathy of undetermined significance turn into?

A

multiple myeloma

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7
Q

What lab technique can you use to diagnose a monoclonal gammopathy?

A

protein electrophoresis

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8
Q

What is a gammopathy exactly?

A

an M spike in the gamma region

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9
Q

What are the immunoglobulins you will most likely see associated with myeloma?

A

IgG and IgA

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10
Q

After the protein electrophoresis, what do you do after to determine what immunoglobulin you’re dealing with?

A

immunofixation

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11
Q

How do you differentiate between a monoclonal gammopathy of undetermined significance and myeloma?

A

% of plasmacytosis in the bone marrow- >60%: multiple myeloma; >10% need more

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12
Q

if the bone marrow is less than 10% plasmacytosis and there is a monoclonal gammopathy, what do you need to diagnose it as a multiple myeloma?

A

CRAB criteria

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13
Q

what are the crab criteria?

A

elevated calcium, impaired renal function, anemia, and bone lesions

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14
Q

What is hypercalcemia a product of? and what are the symptoms associated with it?

A

osteolysis; AMS, cardiac rhythmic disturbances, seizures, n/v, constipation

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15
Q

what is the renal insufficiency in the crab criteria due to?

A

largely due to the immunoglobulins that are being filtered here

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16
Q

In the kidney, immunoglobulin proteins combine with what to form dense CASTS?

A

renal glycoproteins (Tamm Horsfall proteins)

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17
Q

What happens to the dense CASTS that form and what are they referred to as?

A

they clog the tubules and damage the tubular epithelium; known as bence-jones proteins

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18
Q

what happens when the bence-jones proteins damage the renal tubular epithelium?

A

the light chains may be “spilled” into the urine and proteinuria results

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19
Q

What is one final result of renal insufficiency that could occur in myeloma?

A

systemic amyloidosis

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20
Q

What is the type of amyloidosis that may occur in myeloma?

A

AL amyloid (light chain)

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21
Q

Why does anemia occur in myeloma?

A

as a result of marrow infiltration of myeloma; IL-6 and other suppressive cytokines; renal disease leading to decreased erythropoietin

22
Q

Which CRAB manifestations occurs most frequently of all the CRAB manifestations?

A

anemia

23
Q

How do the bone lesions look in the CRAB manifestations in a myeloma patient?

A

punched out appearance

24
Q

In addition to the CRAB manifestations, what could be another result/manifestation of myeloma?

A

hyperviscosity

25
Q

What are some complications associated with hyperviscosity? (2)

A

thromboembolic complications (stroke or venous thrombosis), rouleaux formation

26
Q

What is difference between Waldenstrom’s macroglobulinemia and monocloncal gammopathy of undetermined significance?

A

you still see the gammopathy but with Waldenstrom’s macroglobulinemia you would see settling in the IgM region

27
Q

What is the neoplastic entity associated with Waldenstrom’s macroglobulinemia?

A

LPL (lymphoplasmositic lymphoma)

28
Q

what is the histology like of lymphoplasmacytic lymphoma?

A

b-cell clonal neoplasm of predominant small mature B cells; plasmacytic differentiation

29
Q

What two things are associated with waldenstrom’s macroglobulinemia?

A

monoclonal gammopathy (IgM) and hyperviscosity

30
Q

What are the clinical manifestations/presentations associated with waldenstrom’s macroglobulinemia and hyperviscosity? (3)

A

visual impairment, neurologic manifestations, cryoglobulinemia (ischemia to fingers)

31
Q

What is the main difference between Hodgkin Lymphoma and Non-Hodgkin Lymphoma?

A

hodgkin lymphoma is more often localized to a single axial group of nodes and non-hodgkin lymphoma is more frequent involvement of multiple peripheral nodes

32
Q

How can Hodgkin lymphoma be separated?

A

into classic hodgkin lymphoma and nodular lymphocyte predominant

33
Q

What is the classic Hodgkin lymphoma histologically associated with?

A

the Reed-Sternberg cell, which are multinucleated cells with really prominent nucleoli

34
Q

Before Reed described it, Hodgkin Lymphoma was sometimes diagnostically overlapped with tuberculosis. Why?

A

massive mediastinal lymphadenopathy occurs in tuberculosis as well and you get multinucleated giant cells as some of that granuloma formation

35
Q

What are Reed Sternberg cells positive for?

A

CD15, CD30, and Pax-5

36
Q

What stages is Hodgkin’s lymphoma typically diagnosed in?

A

stages I or II

37
Q

instead of Reed-Sternberg cells, what does nodular lymphocyte predominant hodgkin lymphoma have?

A

popcorn cells (lymphohistiocytic variants (LH cells) )

38
Q

What is Nodular LP Hodgkin Lymphoma positive and negative for?

A

positive for CD20 and negative for CD15 and CD30

39
Q

what can nodular LP Hodgkin lymphoma transform into?

A

DLBCL

40
Q

How do you make the diagnosis of Hodgkin Lymphoma?

A

morphology and staining; it is not typically amenable to flow cytometry;

41
Q

why is Hodgkin lymphoma not typically amenable to flow cytometry?

A

cells constituting the neoplastic clone are too diluted by other cells- so we just look and stain

42
Q

What are three examples of T-cell lymphomas?

A

Anaplastic Large Cell Lymphoma (ALCL), Adult T cell leukemia/lymphoma, Mycosis fungoides and Sezary syndrome

43
Q

who has a better prognosis when diagnosed with anaplastic large cell lymphoma (ALCL)?

A

those that are ALK positive- there is a rearranged ALK gene and when the patients are younger

44
Q

who has a worse prognosis when diagnosed with anaplastic large cell lymphoma (ALCL)?

A

those that are ALK negative and older patients

45
Q

What is the Adult T cell leukemia/lymphoma associated with?

A

HTLV-1 infection

46
Q

what do the cells look like in adult T cell leukemia?

A

cloverleaf cells

47
Q

what is a common clinical presentation of adult T cell leukemia/lymphoma?

A

skin involvement- ranging from exfoliation to popular lesions to necrotizing nodules

48
Q

what is mycosis fungoides?

A

T cell lymphoma presenting with cutaneous manifestations: plaque–> tumor

49
Q

What is Sezary syndrome?

A

T cell lymphoma presenting with erythroderma and leukemia

50
Q

What is red man syndrome associated with?

A

Sezary syndrome

51
Q

Which T cell lymphomas are associated with HTLV-1 and which are not?

A

associated with HTLV-1: Adult T cell leukemia

not associated with HTLV-1: mycosis fungoides and Sezary syndrome