Pathology of White Blood Cells, Lymph Nodes, Spleen, and Thymus (Part 3 of 4) Flashcards
What are the three types of plasma cell neoplasia?
multiple/plasma cell myeloma, monoclonal gammopathy of uncertain significance, and Waldenstrom macroglobulinemia
What are 4 features that are consistent with myeloma? (4)
lytic bone lesions, hypercalcemia, renal failure, immune abnormalities
What is myeloma?
the primary tumor of bone marrow
Histologically speaking, what is the predominant feature of myelomas?
plasma cells- but these are clones
What stains can you use to diagnose myelomas?
CD138, Kappa, and Lambda
What can a monoclonal gammopathy of undetermined significance turn into?
multiple myeloma
What lab technique can you use to diagnose a monoclonal gammopathy?
protein electrophoresis
What is a gammopathy exactly?
an M spike in the gamma region
What are the immunoglobulins you will most likely see associated with myeloma?
IgG and IgA
After the protein electrophoresis, what do you do after to determine what immunoglobulin you’re dealing with?
immunofixation
How do you differentiate between a monoclonal gammopathy of undetermined significance and myeloma?
% of plasmacytosis in the bone marrow- >60%: multiple myeloma; >10% need more
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?
CRAB criteria
what are the crab criteria?
elevated calcium, impaired renal function, anemia, and bone lesions
What is hypercalcemia a product of? and what are the symptoms associated with it?
osteolysis; AMS, cardiac rhythmic disturbances, seizures, n/v, constipation
what is the renal insufficiency in the crab criteria due to?
largely due to the immunoglobulins that are being filtered here
In the kidney, immunoglobulin proteins combine with what to form dense CASTS?
renal glycoproteins (Tamm Horsfall proteins)
What happens to the dense CASTS that form and what are they referred to as?
they clog the tubules and damage the tubular epithelium; known as bence-jones proteins
what happens when the bence-jones proteins damage the renal tubular epithelium?
the light chains may be “spilled” into the urine and proteinuria results
What is one final result of renal insufficiency that could occur in myeloma?
systemic amyloidosis
What is the type of amyloidosis that may occur in myeloma?
AL amyloid (light chain)
Why does anemia occur in myeloma?
as a result of marrow infiltration of myeloma; IL-6 and other suppressive cytokines; renal disease leading to decreased erythropoietin
Which CRAB manifestations occurs most frequently of all the CRAB manifestations?
anemia
How do the bone lesions look in the CRAB manifestations in a myeloma patient?
punched out appearance
In addition to the CRAB manifestations, what could be another result/manifestation of myeloma?
hyperviscosity
What are some complications associated with hyperviscosity? (2)
thromboembolic complications (stroke or venous thrombosis), rouleaux formation
What is difference between Waldenstrom’s macroglobulinemia and monocloncal gammopathy of undetermined significance?
you still see the gammopathy but with Waldenstrom’s macroglobulinemia you would see settling in the IgM region
What is the neoplastic entity associated with Waldenstrom’s macroglobulinemia?
LPL (lymphoplasmositic lymphoma)
what is the histology like of lymphoplasmacytic lymphoma?
b-cell clonal neoplasm of predominant small mature B cells; plasmacytic differentiation
What two things are associated with waldenstrom’s macroglobulinemia?
monoclonal gammopathy (IgM) and hyperviscosity
What are the clinical manifestations/presentations associated with waldenstrom’s macroglobulinemia and hyperviscosity? (3)
visual impairment, neurologic manifestations, cryoglobulinemia (ischemia to fingers)
What is the main difference between Hodgkin Lymphoma and Non-Hodgkin Lymphoma?
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
How can Hodgkin lymphoma be separated?
into classic hodgkin lymphoma and nodular lymphocyte predominant
What is the classic Hodgkin lymphoma histologically associated with?
the Reed-Sternberg cell, which are multinucleated cells with really prominent nucleoli
Before Reed described it, Hodgkin Lymphoma was sometimes diagnostically overlapped with tuberculosis. Why?
massive mediastinal lymphadenopathy occurs in tuberculosis as well and you get multinucleated giant cells as some of that granuloma formation
What are Reed Sternberg cells positive for?
CD15, CD30, and Pax-5
What stages is Hodgkin’s lymphoma typically diagnosed in?
stages I or II
instead of Reed-Sternberg cells, what does nodular lymphocyte predominant hodgkin lymphoma have?
popcorn cells (lymphohistiocytic variants (LH cells) )
What is Nodular LP Hodgkin Lymphoma positive and negative for?
positive for CD20 and negative for CD15 and CD30
what can nodular LP Hodgkin lymphoma transform into?
DLBCL
How do you make the diagnosis of Hodgkin Lymphoma?
morphology and staining; it is not typically amenable to flow cytometry;
why is Hodgkin lymphoma not typically amenable to flow cytometry?
cells constituting the neoplastic clone are too diluted by other cells- so we just look and stain
What are three examples of T-cell lymphomas?
Anaplastic Large Cell Lymphoma (ALCL), Adult T cell leukemia/lymphoma, Mycosis fungoides and Sezary syndrome
who has a better prognosis when diagnosed with anaplastic large cell lymphoma (ALCL)?
those that are ALK positive- there is a rearranged ALK gene and when the patients are younger
who has a worse prognosis when diagnosed with anaplastic large cell lymphoma (ALCL)?
those that are ALK negative and older patients
What is the Adult T cell leukemia/lymphoma associated with?
HTLV-1 infection
what do the cells look like in adult T cell leukemia?
cloverleaf cells
what is a common clinical presentation of adult T cell leukemia/lymphoma?
skin involvement- ranging from exfoliation to popular lesions to necrotizing nodules
what is mycosis fungoides?
T cell lymphoma presenting with cutaneous manifestations: plaque–> tumor
What is Sezary syndrome?
T cell lymphoma presenting with erythroderma and leukemia
What is red man syndrome associated with?
Sezary syndrome
Which T cell lymphomas are associated with HTLV-1 and which are not?
associated with HTLV-1: Adult T cell leukemia
not associated with HTLV-1: mycosis fungoides and Sezary syndrome
