Plasma Cell Disorders Flashcards
Multiple myeloma arises from
Post germinal cells
Multiple myeloma is type of
Plasma cancer
Mutations in plasma cell in case of Multiple myeloma
Chr. 13q deletion (MC)
t(11;14) - Cyclin D1 overexpression
Increased Myc
Chr 17p deletion
Formation of abnormal antibodies is seen in which plasma cancer
Multiple myeloma
Increased no. Of plasma cells can
1) Replace normal BM cells (decreased RBC,WBC And platelets)
2) can lead to Cytokines secretion
Cytokines secreted by plasma cells
IL-6, TNF-alpha, MIP, DKK-4
Cytokines secreted by plasma cells
IL-6, TNF-alpha, MIP, DKK-4
Cytokines secretion from plasma cells can lead to
Increased activity of Osteoclast whereas osteoblasts normal - Results in Bone destruction - Lytic lesions
Most commonly affected bones in multiple myeloma
Vertebral bones > Ribs > Sternum > Pelvis > skull
Most commonly affected bones in multiple myeloma
Vertebral bones > Ribs > Sternum > Pelvis > skull
Serum calcium levels in Multiple myeloma
Increased - Hypercalcemia
Serum alkaline phosphatase levels in Multiple myeloma
Normal - bcz it depends on osteoblasts activity
Hypercalcemia in Multiple myeloma can leads to
Polyuria
Renal failure
2nd most common cause of death in multiple myeloma
Renal failure
Clinical features in multiple myeloma
Bony pain
Common antibody affected in multiple myeloma
IgG
Abnormal M proteins can stick to
RBCs - leads to aggregation of RBC - ROULEAU FORMATION
Increased viscosity of blood in Multiple myeloma can lead to involvement of
Brain - Confusion, dizziness
Increased viscosity of blood in Multiple myeloma can lead to involvement of
Brain - Confusion, dizziness
Due to presence of light chains in urine we can see
Bence jones Proteins
Most common cause of death in multiple myeloma
Severe infection
IMWG updated criteria for multiple myeloma
Clonal BM plasma cells >10% / Biopsy proven Bony extra medullary plasmacytoma
AND
Any 1 of myeloma defining event(MDE)
Myeloma defining events includes
Related organ/ Tissue involvement (ROTI)
Biomarkers of malignancy
Related organ/ tissue impairment includes
Ca2+ levels high - >11mg/dl
Renal dysfunction - s. Creatinine >2mg/dl
Anemia - Hb <10g/dl
Bony lesions - >1 osteolytic lesions
Biomarkers of malignancy includes
BM clonal plasma cells >60%
Light chain - Ratio of involved LC is to Uninvolved LC >100
On MRI - >1lesion (>5mm in size)
Morphological finding in BM Biopsy in MM
MOTT cell
Flame cell (Orangish red color)
Russell Body
Dutcher body
Flow cytometry results in Multiple myeloma
CD19, CD38, CD45, CD138
In Multiple myeloma there is higher expression of which gene
Cyclin D1
Blood examination results in Multiple myeloma
Anemia, Neutropenia, Thrombocytopenia
Increased ESR
Hypercalcemia
Normal serum alkaline phosphatase
Increased levels of Serum beta2 microglobulin and Serum IL-6 levels
Spike seen on Serum protein Electrophoresis
‘M’ Spike
Result of urine examination in multiple myeloma
Presence of Bence Jones proteins
At room temperature urine in presence of Bence Jones proteins
Urine will be clear
At room temperature urine in presence of Bence Jones proteins
Urine will be clear
After heating urine at 40-60°C Urine will be
Turbid urine - because Bence Jones proteins becomes insoluble and coagulates
Urine on heating more than >60°C
Urine again becomes clear
Radiological examination in multiple myeloma
PET Scan
X ray
“Punched out” lesions on skull is seen in
Multiple myeloma
Treatment of Multiple myeloma
Dexamethasone + Lenalidomide + Bortezomib
Diagnostic criteria for Plasma cell Leukemia
Peripheral blood - >20% no. Of plasma cells
Absolute count of plasma cells >2000/microlitre
Good prognosis of multiple myeloma in case of
t(11;14)
Bad prognosis indicators in multiple myeloma
Chr. 17p deletion
Myc overexpression
CRAB +ve
Serum beta2 microglobulin levels high
Best bad prognosis indicator of Multiple myeloma
Serum beta2 microglobulin elevated levels
Differential diagnosis of Multiple myeloma with Monoclonal gammopathy of unknown significance
> 50yr(1%), >70yr(5%)
Clonal plasma cells <10%
No myeloma defining events
Differential diagnosis of multiple myeloma with. Smoldering myeloma
Asymptomatic myeloma
BM plasma cells 10-59%
No myeloid defining events/ No amyloid deposition
Biopsy proven single lesion plasma tumor
Solitary plasmacytoma
Solitary plasmacytoma involves which organs
Bones - same as MM
Soft tissues - Lungs/ paranasal sinuses/oropharynx
Solitary plasmacytoma involves which organs
Bones - same as MM
Soft tissues - Lungs/ paranasal sinuses/oropharynx
Bone marrow in Solitary plasmacytoma
Normal (No tumor cell)
Skeletal screening in Solitary plasmacytoma
Normal ( only 1 lesion)
Solitary plasmacytoma can progress to
Multiple myeloma in 10-20 years
Treatment of Solitary plasmacytoma
Radiotherapy
Surgical resection
Lymphoplasmacytic Lymphoma means
Excessive proliferation of Lymphocytes/plasma cells or increased mast cells
Mutations seen in lymphoplasmacytic Lymphoma
MYD88 gene
Which antibody involved in Lymphoplasmacytic Lymphoma
IgM antibody - Macroglobulinemia - Waldenstroms
Lymphoid tissue involved in Lymphoplasmacytic Lymphoma
Lymph node, spleen, liver
Treatment of Lymphoplasmacytic Lymphoma
Plasmapheresis - abnormal antibodies are removed
Rituximab - against CD20
Abundant chain in heavy chain disease
Heavy chains
Disease due to excessive alpha heavy chains
Seligmann disease
Most common heavy Chain disease
Seligmann disease
Seligmann disease affects which organs
GIT
Respiratory tract
Clinical features of Seligmann disease
Malabsorption syndrome
Weight loss
Diarrhea
Excessive mu heavy chains is associated with
CLL
Light chains may appear in which heavy chain disease
Mu-HCD - Bence Jones proteins++
Light chains may appear in which heavy chain disease
Mu-HCD - Bence Jones proteins++
Disease caused by excessive gamma heavy chains
Franklin’s disease
Clinical features in Franklins disease
Fever
Lymphadenopathy
Hepato and splenomegaly
Franklin’s disease is associated with which disease
Rheumatoid arthritis
