💥What is Monoclonal Gammopathy and when does it’s presence indicate presence of cancer?
💥What is MGUS and MGRS?
#Onconephrology
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💥What is MGUS and MGRS?
#Onconephrology
1/
💥Monoclonal gammopathy is presence of monoclonal immunoglobulin (Ig) in serum, urine or both
💥Monoclonal immunoglobulins are produced by either clonal Plasma cells (often) or by
B lymphocytes (less often)
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💥Monoclonal immunoglobulins are produced by either clonal Plasma cells (often) or by
B lymphocytes (less often)
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⚡️Hematologic criteria for diagnosis of cancer in the setting of monoclonal gammopathy is based on 2 factors:
1. Tumor burden (clonal mass)
2. End-organ damage
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1. Tumor burden (clonal mass)
2. End-organ damage
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⚡️The significance of this criteria is that treatment is generally only recommended for patients who meet the criteria for cancer which is based on the ‘tumor burden’ and presence of ‘end organ damage’
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⚡️For example the diagnostic criteria for Multiple Myeloma:
A) Tumor burden: >10% plasma cells in the bone marrow OR monoclonal spike of >3 g/dl
PLUS
B) End organ damage (CRAB: hypercalcemia, renal failure, anemia, bone lesions)
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A) Tumor burden: >10% plasma cells in the bone marrow OR monoclonal spike of >3 g/dl
PLUS
B) End organ damage (CRAB: hypercalcemia, renal failure, anemia, bone lesions)
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⚡️Smoldering Multiple Myeloma:
A) Tumor burden: >10% clonal plasma cells in bone marrow or a monoclonal spike of >3g/dl
BUT
B) No end-organ damage
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A) Tumor burden: >10% clonal plasma cells in bone marrow or a monoclonal spike of >3g/dl
BUT
B) No end-organ damage
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⚡️CLL (Chronic Lymphocytic Leukemia)
A) Tumor burden: lymphocytosis with >5000 leukemic cells/mm3
PLUS
B) End-organ damage:
-Anemia &/or Thrombocytopenia
OR
-Symptomatic lymphadenopathy &/or Splenomegaly
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A) Tumor burden: lymphocytosis with >5000 leukemic cells/mm3
PLUS
B) End-organ damage:
-Anemia &/or Thrombocytopenia
OR
-Symptomatic lymphadenopathy &/or Splenomegaly
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⚡️Low-grade CLL:
A) Tumor burden: lymphocytosis with >5000 leukemic cells/mm3
BUT
B) No end-organ damage
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A) Tumor burden: lymphocytosis with >5000 leukemic cells/mm3
BUT
B) No end-organ damage
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⚡️Lymphoplasmacytic lymphoma (Waldenstrom’s
macroglobulinemia)
A) Tumor burden: 10% or > lymphocytes with lymphoplasmacytic features in the bone marrow
PLUS
B) End-organ damage: anemia or thrombocytopenia or cryoglobulinemia or hyperviscosity syndrome
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macroglobulinemia)
A) Tumor burden: 10% or > lymphocytes with lymphoplasmacytic features in the bone marrow
PLUS
B) End-organ damage: anemia or thrombocytopenia or cryoglobulinemia or hyperviscosity syndrome
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⚡️Smoldering lymphoplasmacytic lymphoma
A) Tumor burden: 10% or > lymphocytes with lymphoplasmacytic features in the bone marrow
BUT
B) No end-organ damage
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A) Tumor burden: 10% or > lymphocytes with lymphoplasmacytic features in the bone marrow
BUT
B) No end-organ damage
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⚡️MGUS (monoclonal gammopathy of undetermined significance)
-Monoclonal protein is present in serum, urine or both BUT neither the tumor burden criteria outlined above is met nor there is presence of end-organ damage
-No Rx is indicated for MGUS
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-Monoclonal protein is present in serum, urine or both BUT neither the tumor burden criteria outlined above is met nor there is presence of end-organ damage
-No Rx is indicated for MGUS
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⚡️So what if the hematologic criteria for cancer is not met in the setting of a monoclonal gammopathy but the patient has kidney injury due to the underlying monoclonal protein
-This condition is called MGRS
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-This condition is called MGRS
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⚡️MGRS (monoclonal gammopathy of renal significance)
-It represents any Plasma cell or B-cell clonal disorder that does not meet the criteria for cancer but produces monoclonal immunoglobulins that cause kidney disease
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-It represents any Plasma cell or B-cell clonal disorder that does not meet the criteria for cancer but produces monoclonal immunoglobulins that cause kidney disease
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⚡️Treatment of MGRS is generally determined by the nature of the clone producing the nephrotoxic immunoglobulin
-Bortezomib-based therapy for plasma cell clones
-Rituximab-based therapy for B-cell clones that express CD-20
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-Bortezomib-based therapy for plasma cell clones
-Rituximab-based therapy for B-cell clones that express CD-20
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⚡️Significance of MGRS
1. MGRS does not respond well to traditional immunosuppressive therapies used for other nephropathies
2. Untreated MGRS has v. high risk of recurrence post-kidney transplant
3. MGRS patients are at risk for hematologic cancer
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1. MGRS does not respond well to traditional immunosuppressive therapies used for other nephropathies
2. Untreated MGRS has v. high risk of recurrence post-kidney transplant
3. MGRS patients are at risk for hematologic cancer
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