Plasma Cell Disorders Flashcards
Plasma Disorders?
disorders where proliferation and accumulation of Ig secreting cells derived from B lymphocytes
What type of protein do all plasma disorders create that can be seen on electrophoresis?
M protein
What is Monoclonal gammopathy of undetermined significance (MGUS) and what is the criteria?
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
What are people with MGUS at risk of developing?
monoclonal disease,
what are predictors of progression of MGUS?
age, sex, size of M-protein, type of immunoglobulins, hemoglobin, # of bone marrow plasma cells, reduction of uninvolved ig’s, urinary light chains
What are major diagnostic criteria for Multiple myeloma?
biopsy-proven plasmacytoma, bone marrow sample 30% plasma, elevated monoclonal immunoglobulin levels in blood and urine.
What is multiple myeloma?
malignant plasma cell disorder affecting the bone marrow.
What are minor diagnostic criteria for multiple myeloma?
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
what was probably the first tumor marker discovered?
Bence Jones Proteins
How often do radiographs show abnormal finding in patients with multiple myeloma?
80% of patients, osteopenia or osteoporosis, focal lytic bone, pathologic fractures, vertebral body compression fractures.
are spinal MRI effective at detecting MM?
yes, 95% of patients with MM have MRI abnormalities-diffuse BM involvement, focan bone marrow lesions, heterogeneous bone marrow
what 2 ways does MM cause osteiolytic lesions?
stimulation of osteoclasts and inhibition of osteoblasts
MM causes spinal cord compression in 5% of patients, how is this managed?
corticosteroids, neurosurgical intervention + radiation therapy to preserve neurological function
what type of anemia is common in 75% of patients with MM?
normochromic/normocytic anemia
How can MM affect the kidneys?
it can cause renal disease with serum creatinine increased in >50% of patients and 20% have >2g/dl
How can MM cause renal disease?
myeloma cast nephropathy, hypercalcemia, amyloidosis(build up of light chains), radiocontrast dye in patient with myeloma
What is SMM (smoldering multiple myeloma) and what is the criteria?
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
what is the initial approach to MM treatment?
not transplant candidate-melphalan based combinations
transplant candidate-non-alkylator based induction- stem cell harvest
what is therapy for MM?
biphosphonates (pamidronate, zoledronate)-radiotherapy-conventional dose chemo-myeloablative therapy and stem cell transplantation
what are the pros/cons of autografting/allografting stem cell treatment for MM?
autografting-survival improved, but relapse inevitable
allografting-cure is possible, but transplant-related mortality is high
what is Waldenstrom’s macroglobulinemia?
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
how to confirm Waldenstrom
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.
What is polycythemia vera?
raised packed cell volume (HCT) male >51% and femal >48%
clonal stem cell disorder characterised by increased red cell production
What are the disease phases of PV?
proliferative phase, “spent” post-polycythaemic phase, rarely transformed into acute leukemia
What is the most prevalent group of people with PV and what is the most common presenting complications?
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
What are lab features of polycythaemia vera?
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
what is the treatment for polycythaemia vera
to decrease HCT, venesection, chemotherapy-hydroxyurea, low dose aspirin, therapy to reduce itching, antihistamines, H2 blockers or U/V light treatment
what are some causes of secondary polycythaemia vera?
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
what are some tests for secondary PV?
arterial blood gas, Hb electrophoresis, oxygen dissociation curve, EPO, ultrasound abdomen, chest x ray, total red cell volume
What is relative polycythaemia vera?
Caused by loss of plasma and increased relative Hct level, causes-stress, smoking/ETOH, dehydration, plasma loss (burns common)
What is myelofibrosis?
progressive fibrosis of the marrow and increase connective tissue element, extramedullary erythropoiesis (cell production outside of the BM, spleen, liver), abnormal megakaryocytes
What are some symptoms of myelofibrosis?
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
What are some labe presentations of myelofibrosis?
anemia, high WBC at presentation, later leucopenia and thrombocytopenia, leucoerythroblastic blood film, tear drop red cells