Plasma cell dyscrasias Flashcards Preview

Heme/Lymph > Plasma cell dyscrasias > Flashcards

Flashcards in Plasma cell dyscrasias Deck (19):
1

What are the reactive plasma cell conditions?

Chronic infections (H. pylori gastritis, osteomyelitis, endometris)
Autoimmune processes (lupus, hepatitis)

2

What are the plasma cell neoplastic conditions?

Multiple myeloma
Monoclonal gammopathy of undetermined significance (MGUS)
Plasmacytoma (Extramedullary or solitary of bone)
Lymphoplasmatic lymphoma
Amyloidosis

3

How do we assess clonality of plasma cell?

Monoclonal antibodies in the serum or urine
Light chain restriction in cell cytoplasm (kappa v lambda) by flow cytometry or immunohistochemistry
Immunophenotypic aberrancy (e.g. CD56 on plasma cells)

4

What are M proteins?

How do they appear in serum?

How do they appear in urine?

What else can they be assocciated with?

Monoclonal antibodies

Intact immunoglobulins and free light chains

Full antibodies are filitered with kidney damage, free light chains may pass through glomerulus (Bence-Jones proteins

B-cell lymphomas (monoclonal gammopathy), rarely reactive states

5

How are M proteins IDed?

Most important spikes in electrophoresis?

What does a gamma-globulin spike indicate?

What does the immunofixation do?

Identification with electrophoresis and immunofixation

Most important spikes in electrophoresis are the albumin fraction (positive lead) and gamma fraction (negative lead)

Spike means that there is one dominant Ab

Allows specific ID of the gammaglobulin

A image thumb
6

What is multiple myeloma?

Benign/malignant?

Male or female dominant?

Race dominant?

Age range?

Most common plasma cell neoplasm

Malignant

Males greater than female

AAs greater than caucasians

50-70 y/o

7

What are the diagnostic criteria for MM?

Clonal plasma cells - Bone marrow and plasmacytoma

M protein - Serum and/or Urine
Need to check for Bence Jones protein (kidney disease results in complete excretion, 20% have no serum findings)

End organ damage: hyperCalcemia, Renal insufficiency, Anemia, Bone Disease (CRAB

8

Which bone lesions are associated with MM?

Why is there an association with hypercalcemia?

Bone pain
Pathologic fractures in Vertebrae, Ribs, Skull, Pelvis, Femur
Osteopenia

Hypercalcemia is secondary to the bone resorption

9

What  is this?

Q image thumb

Clonal plasma cells in myeloma

10

What effect does M protein have on immune system?

Immunosupression

Elevated risk for recurrent infections due to abnormal Ig production

Most common cause of death

11

What is the prognosis of multiple myeloma?

How is it treated?

Incurable, median survival of 4-6 years treateed (chemo and tandem autologous bone marrow transplants)

12

What is a plasmacytoma?

How is it treated when distinct (non-systemic)?

Are there clonal plasma cells in marrow? M protein?

Localized growth of monoclonal plasma cell

Radiation therapy

No clonal plasma cells in marrow.

+/- M protein

13

What is lymphoplasmacytic lymphoma?

What protein is associated with it?

What syndrome is associated with it?

Lymphoma with plasmacytic differentiation (B cells and plasma cells are neoplastic)

IgM paraprotein

Waldenstrom's macroglobulinemia

14

What are the symptoms of Waldenstrom's macroglobulinemia?

Visual/neurologic impairment
Cryoglobulinemia - Raynaud phenomenon, Bleeding

15

Monoclonal Gammopathy of Undetermined Significance

What is it?

What is the diagnostic criteria?

Most common form of monoclonal gammopathy (2% in >50y/o; 3% in >70y/o)

Diagnostic criteria:
<3 g/dL serum M protein
<10% clonal plasma cells in bone marrow
No myeloma-related end organ damage

16

What is the progression of MGUS?

Which specific diseases can it result in?

What is the follow-up?

"Benign" plasma cell proliferation, but precursor lesion

25% develop malignant transformation over 10-20 year

Multiple myeloma >>>> Lymphoplasmacytic lymphoma +/- Waldenstrom's macroglobulinemia > Primary amyloidosis

Indefinite follow-up, monitoring of M protein

17

What is amyloidosis?

What is the etiology?

What is amyloid?

Diseases characterized by deposition of amyloid

Disorder of protein misfoldings: inherited and acquired forms

Extracellular, proteinaceous misfolded substance

18

What is the most common forms of amyloidosis?

What are the most common deposits and what do they cause?

Amyloid light chain, amyloid associated, and beta-amyloid

Heart - Conduction abnormalities
Kidney - Glomerular damage, nephrotic syndrome
Liver - Hepatomegaly
Nerves - Polyneuropathy
GI Tract - Malabsorption, diarrhea
Tongue - Macroglossia

19

Clinical categories and subtypes of amyloidosis

Systemic (Generalized):
Primary amyloidosis from multiple myeloma (5-10%), other plasma cell disorders
Secondary amyloidosis from chronic inflammatory conditions
Hemodialysis-associated amyloidosis from chronic renal failure

Hereditary

Localized