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38 Cards in this Set

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Which rearranges first, heavy or light chain?
heavy
A plasma cell proliferative disorder in the BM is called?

in the tissues?
myeloma (multiple lesions - multiple myeloma(MM))

plasmacytoma (isolated lesion)
Where are the immunoglobin proteins found on protein electrophoresis?
gamma region b/c they are the slowest moving
What is the predominant light chain, kappa or lambda?

predominant heavy chain?
kappa (3:1)

IgG
What is a pathologic finding on PE for a patient w/ myeloma?
monoclonal M-spike
What step is done after PE to determine what kind of heavy and light chain are the problem?
immunofixation
Monoclonal Gammopathies of Undetermined Significance, some are thought to be ___ related.
virus

BENIGN increase of monoclonal protein, w/o specific cause

can also be biclonal

10-15% progress to MM
What are the two BENIGN plasma cell lesions?
1) MGUS
2) Polyclonal Gammopathy
What are the three MALIGNANT plasma cell lesions?
1) plasma cell dyscrasia
2) multiple myeloma
3) Waldenstrom (IgM)
Heavy globulins that condense when you lower the temperature.

Causes?
Cryoglobinemia

MM, autoimmune diseases (lupus), infections (Hep C)
Primary amyloidosis: amyloid deposition in organs associated w/ ?
high immunoglobulin protein production
Polyclonal protein increases are primarily ____ in nature.
reactive

bacterial, viral infections
Autoimmune diseases
Monoclonal protein increases are primarily seen in ____.
neoplastic or pre-neoplastic conditions
MC heavy chains secreted in MM?
IgG and IgA
Pure light chain secretions in the urine
Bence Jones proteins

detected w/ dip stick, but dip stick only detects protein so you must ask for BJ detection
Diagnostic Criteria for MM
Clinical - anemia and bone pain
Radiograph - lytic bone lesions or fractures
Biochem - protein and Ca
BM PLASMACYTOSIS OVER 30%
Tissue - BM/tissue biopsy

2 major or one minor and one major needed for diagnosis
What would you see on blood smear of a MM patient?
Rouleaux formation (coinstack of RBCs)

Also see plasma cell w/in BM w/ eccentric nuclei and lighter Golgi
What is the problem w/ Rouleaux formations?
can scew the CBC results, double check by remembering that hemoglobin is 3X RBC and hematocrit is 3X hemoglobin
Pathology seen w/ MM?
1)increased paraproteins
2)BM plasmacytosis
3)anemia, hypercalcemia, osteolytic lesions
4) Ras mutations sometimes seen
5)IL-6 dep. proliferation
6)C-myc and p53 mutations in more aggressive forms
What is the criteria for indolent MM?
same as MM but w/o bone lesions and normal hemoglobin and Ca
Criteria for Smoldering Myeloma?
kinda in between MGUS and MM

criteria same as MGUS w/ higher monoclonal protein (between 10-30%)
What is of upmost importance in MM prognosis?
age and performance status

increased paraprotein and beta 2 microglobulin levels associated w/ poor prognosis

tumor mitotic activity and cellular pleomorphism also suggest poor prognosis
Treatment of MM
chemo and BM transplant

generally bad outcomes
Chronic Lymphoid Leukemias (CLL) are generally ___ grade proliferative condition.

Difference btwn CLL and Small lympocytic leukemia (SLL)?
low

CLL has BM leukemic involvement and SLL has tissue involvement
Which lineage of CLL is more common in US?
B-cell lineage

includes prolymphocytic leukemia (PLL) and hairy cell leukemia (HCL)
T cell lineage inludes ? and are generally more ____.
T cell CLLs and adult T-cell lymphoma/leukemia

aggresive
CLLs usually occur in ____ patients w/ a ____ predominance.
older (>55)

male
CLL Lab findings?
1) striking lymphocytosis
2) mature appearing lymphoid cells
3) smudge cells (increased fragility)
Clinical findings of CLL
1) lymphadenopathy
2) anemia
3) frequent infections
4) autoimmune complications
Markers for B cell CLL?

___ expression is high.
CD5+ and CD20+

Bcl-2, trisomy 12 indicates poor prognosis
What actually kills a CLL patient?
2nd malignancy or a complication from infection/bleeding
CLL progression to high grade lymphoma?
Richter's syndrome
How do you differentiate CLL from PLL and acute lymphocytic leukemia?
PLL has prominent nucleoli

AML has predominant immature blasts, finer chromatin, and are smaller than PLL
What is PLL?
aggressive form of CLL

large cells w/ prominent nucleoli

bright surface immunoglobulin expression, w/ poor prognosis
What are "flower cells" associated w/?
T-cell lymphocytic leukemia
What is Hairy Cell Leukemia?
type of B cell CLL, usually seen in elderly men

associated w/ massive splenomegaly and recurrent oppertunisic infections

Think of a hairy old man who has a huge spleen and is sick.
Smear appearance of HCL?
hair like projections, TRAP stain is positive

in BM, striking reticulin fibers w/ silver stain
Markers for HCL?
dry tap on bone marrow (not sure what that means, probably doesn't matter)

CD25+, CD11c+, CD103+

Treatment: chemo