Protocols - Hemorio

WALDENSTRON MACROGLOBULINEMIA
LABORATORIAL TESTS TO THE DIAGNOSIS:
- Full Hemogram
- Biochemistry with renal and hepatic function proofs
- Coagulogram
- Myelogram with evaluation of the infiltration by lymphocytes
- Immunophenotype
- BMB
- Immunoelectrophoresis
- Dosage of Immunoglobulin IgG, IgA, IgM, mild chains
Diagnosis criteria:
- IgM-value independent Monoclonal Gammopathy IgM
- >10% of infiltration of MO by small lymphocytes that present Plasmocyte differentiation
- Typical immunophenotype (i.e., IgM of surface +, CD5+/-, CD10-, CD19+, CD20+, CD22+, CD23-,
CD25+, CD27+, FMC7+, CD103-, CD138-);
- MW smoldering – Criteria above, in asymptomatic subjects or those with no anemia. They must not be
treated.
- Monoclonal Gammopathy IgM with an indefinite meaning
- Dosage of IgM < 3.0 g/dL
- Absence of anemia, hepatosplenomegaly, lymphadenopathy and systemic symptoms.
- Minimal or no infiltration lymphoplasmacytic of MO (< 10%).
STAGING
- Stage A (low risk) - β2M12 OS in 5 years=87%
- Stage B (mean risk) - β2M4 OS in 5 years=21%
TREATMENT
- Asymptomatic subjects (Smoldering MV) – Must not be treated
- Treatment indications - Symptoms related to the hyperviscosity (oronasal bleeding, blurred eye,
headache, paresthesias, torpor, coma), anemia, pancytopenia, symptomatic organomegaly, bulky, base
lymphoproliferative disease, paraneoplastic neuropathy.
- Plasmapheresis - hyperviscosity, bleeding, neurological setting
- Chemotherapy
- Chlorambucil – 0.1mg/Kg/d continuous usage or 0.3mg/Kg/d for 7 days every 4 to 6 weeks until gets to
the plateau
- CHOP
- Purine Analogous - Cladribine, Fludarabine
- Other regimens - Melphalan (6mg/m2), Cyclophosphamide (125mg/m2) and Prednisone (40mg/m2) D1
to D7 every 4 to 6 weeks up to 12 cycles. When the disease is stable, start Chlorambucil 3mg/m2 and
Prednisone 6mg/m2 daily until the disease progression.
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