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

Question 1

What are the three types of plasma cell neoplasia?

Answer 1

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

Question 2

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

Answer 2

lytic bone lesions, hypercalcemia, renal failure, immune abnormalities

Question 3

What is myeloma?

Answer 3

the primary tumor of bone marrow

Question 4

Histologically speaking, what is the predominant feature of myelomas?

Answer 4

plasma cells- but these are clones

Question 5

What stains can you use to diagnose myelomas?

Answer 5

CD138, Kappa, and Lambda

Question 6

What can a monoclonal gammopathy of undetermined significance turn into?

Answer 6

multiple myeloma

Question 7

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

Answer 7

protein electrophoresis

Question 8

What is a gammopathy exactly?

Answer 8

an M spike in the gamma region

Question 9

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

Answer 9

IgG and IgA

Question 10

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

Answer 10

immunofixation

Question 11

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

Answer 11

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

Question 12

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?

Answer 12

CRAB criteria

Question 13

what are the crab criteria?

Answer 13

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

Question 14

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

Answer 14

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

Question 15

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

Answer 15

largely due to the immunoglobulins that are being filtered here

Question 16

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

Answer 16

renal glycoproteins (Tamm Horsfall proteins)

Question 17

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

Answer 17

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

Question 18

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

Answer 18

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

Question 19

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

Answer 19

systemic amyloidosis

Question 20

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

Answer 20

AL amyloid (light chain)

Question 21

Why does anemia occur in myeloma?

Answer 21

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

Question 22

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

Answer 22

anemia

Question 23

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

Answer 23

punched out appearance

Question 24

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

Answer 24

hyperviscosity

Question 25

What are some complications associated with hyperviscosity? (2)

Answer 25

thromboembolic complications (stroke or venous thrombosis), rouleaux formation

Question 26

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

Answer 26

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

Question 27

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

Answer 27

LPL (lymphoplasmositic lymphoma)

Question 28

what is the histology like of lymphoplasmacytic lymphoma?

Answer 28

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

Question 29

What two things are associated with waldenstrom's macroglobulinemia?

Answer 29

monoclonal gammopathy (IgM) and hyperviscosity

Question 30

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

Answer 30

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

Question 31

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

Answer 31

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

Question 32

How can Hodgkin lymphoma be separated?

Answer 32

into classic hodgkin lymphoma and nodular lymphocyte predominant

Question 33

What is the classic Hodgkin lymphoma histologically associated with?

Answer 33

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

Question 34

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

Answer 34

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

Question 35

What are Reed Sternberg cells positive for?

Answer 35

CD15, CD30, and Pax-5

Question 36

What stages is Hodgkin's lymphoma typically diagnosed in?

Answer 36

stages I or II

Question 37

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

Answer 37

popcorn cells (lymphohistiocytic variants (LH cells) )

Question 38

What is Nodular LP Hodgkin Lymphoma positive and negative for?

Answer 38

positive for CD20 and negative for CD15 and CD30

Question 39

what can nodular LP Hodgkin lymphoma transform into?

Answer 39

DLBCL

Question 40

How do you make the diagnosis of Hodgkin Lymphoma?

Answer 40

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

Question 41

why is Hodgkin lymphoma not typically amenable to flow cytometry?

Answer 41

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

Question 42

What are three examples of T-cell lymphomas?

Answer 42

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

Question 43

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

Answer 43

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

Question 44

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

Answer 44

those that are ALK negative and older patients

Question 45

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

Answer 45

HTLV-1 infection

Question 46

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

Answer 46

cloverleaf cells

Question 47

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

Answer 47

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

Question 48

what is mycosis fungoides?

Answer 48

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

Question 49

What is Sezary syndrome?

Answer 49

T cell lymphoma presenting with erythroderma and leukemia

Question 50

What is red man syndrome associated with?

Answer 50

Sezary syndrome

Question 51

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

Answer 51

associated with HTLV-1: Adult T cell leukemia | not associated with HTLV-1: mycosis fungoides and Sezary syndrome