Plasma Cell Disorders

Question 1

Plasma Disorders?

Answer 1

disorders where proliferation and accumulation of Ig secreting cells derived from B lymphocytes

Question 2

What type of protein do all plasma disorders create that can be seen on electrophoresis?

Answer 2

M protein

Question 3

What is Monoclonal gammopathy of undetermined significance (MGUS) and what is the criteria?

Answer 3

presence of m-protein in a patient without a plasma cell or lymphoproliferative disorder
<10% plasma cells in bone marrow
-no or small amounts of M-protein in urine
-absence of lytic bone lesions, anemia, hypercalcemia or renal insufficiency
-no evidence of B or lymphoproliferative disorder
-stability of M-protein over time
-prevalence increases with age

Question 4

What are people with MGUS at risk of developing?

Answer 4

monoclonal disease,

Question 5

what are predictors of progression of MGUS?

Answer 5

age, sex, size of M-protein, type of immunoglobulins, hemoglobin, # of bone marrow plasma cells, reduction of uninvolved ig’s, urinary light chains

Question 6

What are major diagnostic criteria for Multiple myeloma?

Answer 6

biopsy-proven plasmacytoma, bone marrow sample 30% plasma, elevated monoclonal immunoglobulin levels in blood and urine.

Question 7

What is multiple myeloma?

Answer 7

malignant plasma cell disorder affecting the bone marrow.

Question 8

What are minor diagnostic criteria for multiple myeloma?

Answer 8

bone marrow sample =10% to 30% plasma cells, minor ig levels in blood or urine <3g/dl, osteopenia/lytic bone lesions, abnormally low antibody levels

Question 9

what was probably the first tumor marker discovered?

Answer 9

Bence Jones Proteins

Question 10

How often do radiographs show abnormal finding in patients with multiple myeloma?

Answer 10

80% of patients, osteopenia or osteoporosis, focal lytic bone, pathologic fractures, vertebral body compression fractures.

Question 11

are spinal MRI effective at detecting MM?

Answer 11

yes, 95% of patients with MM have MRI abnormalities-diffuse BM involvement, focan bone marrow lesions, heterogeneous bone marrow

Question 12

what 2 ways does MM cause osteiolytic lesions?

Answer 12

stimulation of osteoclasts and inhibition of osteoblasts

Question 13

MM causes spinal cord compression in 5% of patients, how is this managed?

Answer 13

corticosteroids, neurosurgical intervention + radiation therapy to preserve neurological function

Question 14

what type of anemia is common in 75% of patients with MM?

Answer 14

normochromic/normocytic anemia

Question 15

How can MM affect the kidneys?

Answer 15

it can cause renal disease with serum creatinine increased in >50% of patients and 20% have >2g/dl

Question 16

How can MM cause renal disease?

Answer 16

myeloma cast nephropathy, hypercalcemia, amyloidosis(build up of light chains), radiocontrast dye in patient with myeloma

Question 17

What is SMM (smoldering multiple myeloma) and what is the criteria?

Answer 17

M-protein is >3g/dl, bone marrow plasma cells >10% but asymptomatic with no organ related problems.-requires no intervention but close surveillance for stability

Question 18

what is the initial approach to MM treatment?

Answer 18

not transplant candidate-melphalan based combinations

transplant candidate-non-alkylator based induction- stem cell harvest

Question 19

what is therapy for MM?

Answer 19

biphosphonates (pamidronate, zoledronate)-radiotherapy-conventional dose chemo-myeloablative therapy and stem cell transplantation

Question 20

what are the pros/cons of autografting/allografting stem cell treatment for MM?

Answer 20

autografting-survival improved, but relapse inevitable

allografting-cure is possible, but transplant-related mortality is high

Question 21

what is Waldenstrom’s macroglobulinemia?

Answer 21

high serum IgM, enlargement of spleen, liver, lymph nodes, plasmacytoid infiltration of BM, spleen, nodes, mast cell infiltrates seen, suppression of bone marrow function, hyperviscocity syndrome, cryoglobulinemia, neuropathy

Question 22

how to confirm Waldenstrom

Answer 22

serum protien electrophoresis, immunofixation (characterize light and heavy chains), 24hr urin collection, serum B2 microglobulin, bone marrow biopsy, cytogenetics studies, CT to detect organomegaly and lymphadenopathy, blood or serum viscosity.

Question 23

What is polycythemia vera?

Answer 23

raised packed cell volume (HCT) male >51% and femal >48%

clonal stem cell disorder characterised by increased red cell production

Question 24

What are the disease phases of PV?

Answer 24

proliferative phase, “spent” post-polycythaemic phase, rarely transformed into acute leukemia

Question 25

What is the most prevalent group of people with PV and what is the most common presenting complications?

Answer 25

55-60 YO, males>females, rare in young adults and children

vascular complications-thrombosis, DVT, HTN, HA, poor vision, dizziness, skin complications, hemorrhage due to platelet defect

Question 26

What are lab features of polycythaemia vera?

Answer 26

Hb, HCT and red cell mass increased, increased neutrophils and platelets, low o2 levels, plasma urate high, erythroid precursors, hypercellular bone marrow, low serum erythropoietin

Question 27

what is the treatment for polycythaemia vera

Answer 27

to decrease HCT, venesection, chemotherapy-hydroxyurea, low dose aspirin, therapy to reduce itching, antihistamines, H2 blockers or U/V light treatment

Question 28

what are some causes of secondary polycythaemia vera?

Answer 28

high altitude, pulmonary disease, cyanotic heart disease, abnormal hemoglobin (high affinity hemoglobin), heavy cigarette smoker, innappropriate EPO production-renal disease-carcinoma, hydronephrosis
tumors-fibromyoma and liver carcinoma

Question 29

what are some tests for secondary PV?

Answer 29

arterial blood gas, Hb electrophoresis, oxygen dissociation curve, EPO, ultrasound abdomen, chest x ray, total red cell volume

Question 30

What is relative polycythaemia vera?

Answer 30

Caused by loss of plasma and increased relative Hct level, causes-stress, smoking/ETOH, dehydration, plasma loss (burns common)

Question 31

What is myelofibrosis?

Answer 31

progressive fibrosis of the marrow and increase connective tissue element, extramedullary erythropoiesis (cell production outside of the BM, spleen, liver), abnormal megakaryocytes

Question 32

What are some symptoms of myelofibrosis?

Answer 32

splenomegaly (massive), hypermetabolic symptoms (weight loss, fever, night sweats, myelofibrosis, bleeding problems, bone pain, gout, can transform to acute leukemia in 10-20% of cases

Question 33

What are some labe presentations of myelofibrosis?

Answer 33

anemia, high WBC at presentation, later leucopenia and thrombocytopenia, leucoerythroblastic blood film, tear drop red cells