MM Flashcards
If whole-body low-dose CT or FDG-PET/ CT is negative, consider ____________ to discern smoldering myeloma from multiple myeloma (MM)
Whole-body MRI without contrast
CT contrast agents are not necessary for detection of myeloma bone disease and should generally be avoided in myeloma patients whenever possible.
The FISH panel for prognostic estimation of plasma cells should be examined for
- del(13)
- del(17p13)
- t(4;14)
- t(11;14)
- t(14;16)
- t(14:20)
- 1q21 gain/amplification
- 1p deletion
Primary treatment for: Solitary plasmacytoma or Solitary plasmacytoma with minimal marrow involvement
Solitary plasmacytoma with 10% or more clonal plasma cells is regarded as active (symptomatic) MM and systemic therapy should be considered.
RTm ± surgery
or Consider clinical trial
Systemic therapy may be considered in patients with high risk of progression based on the clinical context.
Follow-up interval for evaluation in: Solitary plasmacytoma or Solitary plasmacytoma with minimal marrow involvement after RT
Every 3–6 mo
All plasmacytomas should be imaged _________ , preferably with the same technique used at diagnosis, for at least 5 years
Yearly
Considered as High risk Smoldering myeloma
- Bone marrow plasma cells (BMPCs) > 20%
- M-protein > 2 g/dL
- Serum FLC ratio (FLCr) > 20
The Mayo 2018/IMWG 20/2/20 criteria
Patients with two or more of these risk factors are considered to have high risk of progression to MM.
Preferred treatment for High risk Smoldering myeloma
3 months
Medication that can be considered for High risk Smoldering myeloma
Lenalidomide
TRUE OR FALSE
Immunofixation is needed only if protein electrophoresis is negative during follow-up.
TRUE
Immunofixation is needed only if protein electrophoresis is negative during follow-up.
TRUE OR FALSE
Renal dysfunction and advanced age are contraindications to transplant.
FALSE
Renal dysfunction and advanced age are not contraindications to transplant.
TRUE OR FALSE
Patients with stable disease can be considered for autologous HCT.
TRUE
Patients with stable disease can be considered for autologous HCT.
Options if there is response after primary therapy
- Autologous HCT(category 1)
- Continuous myeloma therapy or maintenance therapy
- Tandem autologous or allogeneic HCT for patients with high-risk of progression/relapse, under certain circumstances
Criteria
Smoldering Myeloma (Asymptomatic)
- Serum monoclonal protein ≥3 g/dL or Bence-Jones protein ≥500 mg/24 h
and/or - Clonal bone marrow plasma cells (BMPCs) 10%–59%
and - Absence of myeloma-defining events or amyloidosis
Criteria
Multiple Myeloma (Symptomatic)
- Clonal BMPCs ≥10% or biopsy-proven bony or extramedullary plasmacytoma
and
Any one or more of the following myeloma-defining events:
* Calcium > 0.25 mmol/L (>1 mg/dL) higher than the upper limit of normal or > 2.75 mmol/L (>11 mg/dL)
* Renal insufficiency (creatinine > 2 mg/dL) [>177 μmol/L] or creatinine clearance < 40 mL/min
* Anemia (hemoglobin < 10 g/dL or hemoglobin > 2 g/dL below the lower limit of normal)
* One or more osteolytic bone lesions on skeletal radiography, CT, or FDG-PET/CT
* Clonal BMPCs ≥ 60%
* Involved:uninvolved serum FLC ratio (FLCr) ≥ 100 and involved FLC concentration 10 mg/dL or higher
* >1 focal lesions on MRI studies ≥ 5 mm
SLiM-CRAB
Factors Considered as High Risk for Progression/Relapse
For Those with Newly Diagnosed MM
- R-ISS III
- Extramedullary disease
- Circulating plasma cells
- Cytogenetic abnormalities
�Del(1p32)
�t(4;14)
�t(14;16)
�t(14;20)
�Del(17p)/monosomy 17/TP53 mutation
�1q21 gain/1q21 amplificationb
� MYC translocation - High-risk gene expression profile
1q21 gain/amplification alone is not considered high-risk for progression/relapse.
Factors Considered as High Risk for Progression/Relapse
For Those with Relapsed MM
- Disease relapse within 2 years of initial therapy when transplant and maintenance are used.
- Relapse within 18 mo in case of nontransplant–
based treatment. - Acquisition of 1q gain/amplification and/or del(17p)/TP53 mutation
- Extramedullary disease at relapse
Presence of ≥ _____ % of plasma cells in circulation is defined as plasma cell leukemia.
≥5%
International Staging System (ISS)
- I: Serum beta-2 microglobulin <3.5 mg/L, Serum albumin ≥3.5 g/dL
- II: Not ISS stage I or III
- III: Serum beta-2 microglobulin ≥5.5 mg/L
Revised-ISS (R-ISS)
- I: ISS stage I and standard-risk chromsomal abnormalities by FISH and Serum LDH ≤ the upper limit of normal
- II: Not R-ISS stage I or III
- III: ISS stage III and either high-risk chromosomal abnormalities by FISH or Serum LDH > the upper limit of normal
R2-ISS
R2-ISS is only validated for newly diagnosed Multiple Myeloma.
A numerical value is assigned to each risk factor based on their influence on OS:
* ISS-III is 1.5 points
* ISS-II is 1 point
* del(17p) is 1 point
* t(4;14) is 1 point
* 1q+ is 0.5 points and
* serum LDH > the upper limit of normal is 1 point
- Low-risk: 0 points
- Low-intermediate risk: 0.5–1 points
- Intermediate-high risk: 1.5–2.5 points
- High-risk: 3–5 points
Recommended for initial diagnostic workup of patients suspected of having MM or solitary
plasmacytoma
Whole-body low-dose CT or FDG-PET/CT
Skeletal survey is acceptable in certain circumstances.
The first choice for initial evaluation of solitary osseous plasmacytoma
Whole-body MRI (or FDG-PET/CT if MRI is not available)
Wholebody FDG-PET/CT is the first choice for initial evaluation of solitary extraosseous plasmacytoma.
Clinical target colume (CTV) expansions should generally include at least ______ cm of margin for microscopic extent, and up to ____ cm for involvement of long bones.
0.5 cm
2–3 cm
RT dose for solitary plasmacytoma
RT (40–50 Gy in 1.8–2.0 Gy fractions [20–25 total fractions])
Treatment with 35–40 Gy is an acceptable alternative for solitary plasmacytomas < 5 cm in size, due to the high rates of local control reported for smaller tumors.
TRUE OR FALSE
RT is primarily used for palliation in patients with MM.
TRUE
RT is primarily used for palliation in patients with MM.
Systemic therapy should not be delayed for RT, and can often be given concurrently.
Palliative RT Dosing for MM:
Low-dose RT (8 Gy x 1 fraction) or 20–30 Gy in 5–10 total fractions can be used as palliative treatment for uncontrolled pain, for impending pathologic fracture, or for impending cord compression.
Moderately fractionated courses of 20-25 Gy in 8-10 fractions are generally preferred over higher doses (30 Gy) absent extenuating circumstances (e.g., severe symptomatic cord compression) to limit unnecessary toxicity and reduce risk of future treatment of adjacent or overlapping organs at risk (e.g., spinal cord).
Standard IMWG response criteria
Stringent complete response
- Complete response plus
- Normal FLC ratio and
- Absence of clonal cells in bone marrow biopsy by immunohistochemistry (κ/λ ratio ≤4:1 or ≥1:2 for κ and λ patients, respectively, after counting ≥100 plasma cells).
The FLCr is required for documenting sCR according to the IMWG Uniform Response Criteria.
The serum FLC assay cannot replace the 24-hour UPEP for monitoring patients with measurable urinary M protein and can also be affected by renal function.
Standard IMWG response criteria
Complete response
- Negative immunofixation on the serum and urine and
- Disappearance of any soft tissue plasmacytomas and
- <5% plasma cells in bone marrow aspirates
Standard IMWG response criteria
Very good partial response
- Serum and urine M-protein detectable by immunofixation but not on electrophoresis or
- ≥90% reduction in serum M-protein plus urine M-protein level <100 mg per 24 h
Standard IMWG response criteria
Partial response
- ≥50% reduction of serum M-protein plus
- Reduction in 24-h urinary M-protein by ≥90% or to <200 mg per 24 h.
- If the serum and urine M-protein are unmeasurable, a ≥ 50% decrease in the difference between involved and uninvolved FLC levels is required in place of the M-protein criteria.
- If serum and urine M-protein are unmeasurable, and serum-free light assay is also unmeasurable, ≥ 50% reduction in plasma cells is required in place of M-protein, provided baseline bone marrow plasma-cell percentage was ≥30%.
- In addition to these criteria, if present at baseline, a ≥ 50% reduction in the size (sum of the products of the maximal perpendicular diameters [SPD] of measured lesions)j of soft tissue plasmacytomas is also required.
Standard IMWG response criteria
Minimal response
- ≥ 25% but ≤49% reduction of serum M-protein and
- Reduction in 24-h urine M-protein by 50%–89%.
- In addition to the above listed criteria, if present at baseline, a 25%–49% reduction in SPD of soft tissue plasmacytomas is also required.
Standard IMWG response criteria
Stable disease
Not meeting criteria for complete response, very good partial response, partial response, minimal
response, or progressive disease.
Not recommended for use as an indicator of response; stability of disease is best described by providing the time-to-progression estimates
Standard IMWG response criteria
Progressive disease
Any one or more of the following criteria:
* Increase of 25% from lowest confirmed response value in one or more of the following criteria:
>Serum M-protein (absolute increase must be ≥0.5 g/dL);
>Serum M-protein increase ≥1 g/dL, if the lowest M component was ≥5 g/dL;
>Urine M-protein (absolute increase must be ≥200 mg/24 h);
- In patients without measurable serum and urine M-protein levels, the difference between involved and uninvolved FLC levels (absolute increase must be >10 mg/dL);
- In patients without measurable serum and urine M-protein levels and without measurable involved FLC levels, bone marrow plasma-cell percentage irrespective of baseline status (absolute increase must be ≥10%);
- Appearance of a new lesion(s), ≥50% increase from nadir in SPDj of >1 lesion, or ≥50% increase in the longest diameter of a previous lesion >1 cm in short axis;
- ≥ 50% increase in circulating plasma cells (minimum of 200 cells per μL) if this is the only measure of disease
Standard IMWG response criteria
Clinical relapse
- Direct indicators of increasing disease and/or end organ dysfunction (calcium elevation, renal failure, anemia, lytic bone lesions [CRAB features]) related to the underlying clonal plasma cell proliferative disorder.
- Development of new soft tissue plasmacytomas or bone lesions (osteoporotic fractures do not constitute progression);
- Definite increase in the size of existing plasmacytomas or bone lesions.
A definite increase is defined as a 50% (and ≥1 cm) increase as measured serially by the SPD of the measurable lesion; - Hypercalcemia (>11 mg/dL);
- Decrease in hemoglobin of ≥2 g/dL not related to therapy or other non–myeloma-related conditions;
- Rise in serum creatinine by 2 mg/dL or more from the start of the therapy and attributable to myeloma;
- Hyperviscosity related to serum paraprotein
It is not used in calculation of time to progression or progression-free survival but is listed as something that can be reported optionally or for use in clinical practice;
Standard IMWG response criteria
Relapse from complete response
(to be used only if the endpoint is disease-free survival)
Any one or more of the following criteria:
* Reappearance of serum or urine M-protein by immunofixation or electrophoresis;
* Development of ≥5% plasma cells in the bone marrow;
* Appearance of any other sign of progression (ie, new plasmacytoma, lytic bone lesion, or hypercalcemia) (see above).
Standard IMWG response criteria
Relapse from MRD negative
(to be used only if the endpoint is disease-free survival)
Any one or more of the following criteria:
* Loss of MRD negative state (evidence of clonal plasma cells on NGF or NGS, or positive imaging study for recurrence of myeloma);
* Reappearance of serum or urine M-protein by immunofixation or electrophoresis;
* Development of ≥5% clonal plasma cells in the bone marrow;
* Appearance of any other sign of progression (ie, new plasmacytoma, lytic bone lesion, or hypercalcemia).
TRUE OR FALSE
All response categories require two consecutive assessments made any time before starting any new therapy.
TRUE
All response categories require two consecutive assessments made any time before starting any new therapy.
For MRD there is no need for two consecutive assessments, but information on MRD after each treatment stage is recommended (eg, after induction, high-dose therapy/ Autologous stem cell transplants (ASCT), consolidation, maintenance).
*Radiographic studies are not required to satisfy these response requirements. *
*Bone marrow assessments do not need to be confirmed. *
When the only method to measure disease is by serum FLC levels:
- Complete response: a normal FLC ratio of 0.26 to 1.65 in addition to the complete response criteria
- Very good partial response in such patients requires a ≥90% decrease in the difference between involved and uninvolved FLC levels.
Patients should receive at least a ____________ regimen if they can tolerate it.
Triplet regimen (2 drug classes and steroids)
Patients with poor performance status or who are frail can be started on a 2-drug regimen, with a third drug added once performance status improves.
Consider dose modifications based on functional status and age.
For relapsed disease, if relapse is greater than _____ months after the end of primary treatment, the regimen used for primary therapy may be repeated.
6 months
TRUE OR FALSE: R/R Myeloma
A new triplet regimen should preferably include drugs or drug classes patients have not been exposed to, or not exposed to for at least 6 months.
TRUE
A new triplet regimen should preferably include drugs or drug classes patients have not been exposed to, or not exposed to for at least 6 months.
Intravenous immunoglobulin (IVIG) should be considered for patients with an IgG _____ mg/dL prior to the administration of bispecfic T-cell antibodies and CAR T-cell therapy.
IgG <400 mg/dL
Consider harvesting peripheral blood stem cells within the _________ cycles of therapy initiation prior to prolonged exposure to lenalidomide and/or
daratumumab in patients for whom HCT is being considered.
Within the first 6 cycles of therapy
Exposure to myelotoxic agents (including alkylating agents and nitrosoureas) should be limited to avoid compromising stem cell reserve prior to stem cell harvest in patients who may be candidates for HCT.
***notably Melphalan
Preferred method of administration of bortezomib
Subcutaneous
Both weekly and twice-weekly dosing schemas of bortezomib may be appropriate; weekly preferred.
Carfilzomib may be used once or twice weekly and at different doses.
TRUE OR FALSE
Daratumumab and hyaluronidase-fihj for subcutaneous injection has same dosing with daratumumab for intravenous infusion.
FALSE
Daratumumab and hyaluronidase-fihj for subcutaneous injection has different dosing and administration instructions compared to daratumumab for intravenous infusion.
Steroids should be reduced to ________ mg weekly in older patients and should be decreased or discontinued with treatment response plateau and/ or toxicity.
20 mg weekly
Medications that may interfere with serologic testing and cause false-positive indirect Coombs test.
Daratumumab and isatuximab-irfc
Type and screen should be performed before using daratumumab or isatuximab-irfc.
Drug that can produce a false positive serum immunofixation if the monoclonal protein is IgG kappa
Monoclonal antibodies
Special interference testing or mass spectrometry based assessment can differentiate between the two.
Drug that can potentially cause cardiac and pulmonary toxicity, especially in older patients.
Carfilzomib
Drug that can impact the ability to collect T cells for CAR T-cell therapy.
Bendamustine
Preferred Regimens for Transplant Candidates
- Bortezomib/lenalidomide/dexamethasone (category 1)
- Carfilzomib/lenalidomide/dexamethasone
Other Recommended Regimens
* Daratumumab/lenalidomide/bortezomib/dexamethasone
Regimen preferred primarily as initial treatment in patients with acute renal insufficiency or those who have no access to proteasome inhibitor (PI)/lenalidomide/ dexamethasone.
- Bortezomib/cyclophosphamide/dexamethasone
- Carfilzomib/cyclophosphamide/dexamethasone
Consider switching to PI/lenalidomide/dexamethasone after renal function improves.
Treatment option for patients with renal insufficiency and/or peripheral neuropathy.
Carfilzomib/cyclophosphamide/dexamethasone
Generally reserved for the treatment of aggressive MM
Dexamethasone/thalidomide/cisplatin/doxorubicin/cyclophosphamide/etoposide/bortezomibg (VTD-PACE)
Drug that appears to have an increased risk for secondary cancers, especially with maintenance following transplant.
.
Lenalidomide
The benefits and risks of maintenance therapy vs. secondary cancers should be discussed with patients
Preferred Regimens for Maintainance-Transplant Candidates
Lenalidomide (category 1)
Other Recommended Regimens: Bortezomib
Two drug maintenance recommended for high-risk MM
Results from interim analyses of TOURMALINE MM3 and MM4 trials of ixazomib in the maintenance setting suggest a potential decrease in overall survival (OS).
Patients with high-risk cytogenetics have been shown to benefit from a combination of PI and IMiD as maintenance therapy
Refers to a planned second course of high-dose therapy and HCT within 6 months of the first course
An option for patients who do not achieve at least a VGPR after the first autologous HCT and those with high-risk features
Tandem HCT
The NCCN Multiple Myeloma Panel recommends collecting enough hematopoietic stem cells for at least one HCT in all eligible patients, and for two transplants in the younger patients if tandem transplant or repeat transplant would be considered.
Preferred Regimens for Non-Transplant Candidates
- Bortezomib/lenalidomide/dexamethasone (category 1)
- Daratumumab/lenalidomide/dexamethasone (category 1)
Other Recommended Regimens:
* Daratumumab/bortezomib/melphalan/prednisone (category 1)
* Carfilzomib/lenalidomide/dexamethasone
* Daratumumab/cyclophosphamide/bortezomib/dexamethasone
Regimen for Non-Transplant Candidates that can be given continuously until progression
Lenalidomide/low-dose dexamethasone (category 1)
Regimen for Non-Transplant Candidates that can be given for frail patients
Bortezomib/lenalidomide/dexamethasone (VRD-lite)
Preferred Regimens for Maintainance-Non-Transplant Candidates
Lenalidomide (category 1)
Other Recommended Regimens: Bortezomib
Patients with high-risk cytogenetics have been shown to benefit from a combination of PI and IMiD as maintenance therapy
CAR T-Cell Therapy for MM
- Ciltacabtagene autoleucel (category 1)- After one prior therapy including IMiD and a PI, and refractory to lenalidomide
- Idecabtagene vicleucel (category 1)- After two prior therapies including an IMiD, an anti-CD38 monoclonal antibody and a PI
Preferred Regimens for Relapsed/Refractory Disease After 3 Prior Therapies
After at least four prior therapies, including an anti-CD38 monoclonal antibody, a PI, and an IMiDs
CAR T-cell Therapy:
◊ Ciltacabtagene autoleucel
◊ Idecabtagene vicleucel
Bispecific Antibodies:
◊ Elranatamab-bcmm
◊ Talquetamab-tgvs
◊ Teclistamab-cqyv
Other Recommended Regimens:
* Bendamustine
* Bendamustine/bortezomib/dexamethasone
* Bendamustine/carfilzomib/dexamethasone
* Bendamustine/lenalidomide/dexamethasone
* High-dose or fractionated cyclophosphamide
Can be used after at least three prior therapies including a PI and an IMiD or are doublerefractory
to a PI and an IMiD
Daratumumab
Can be used for t(11;14) patients
Venetoclax/dexamethasone ± daratumumab or PI only
Can be used after at least four prior therapies, including an anti-CD38 monoclonal antibody, a PI, and an IMiD
Selinexor/dexamethasone
Belantamab mafodotin-blmf
A BCMA antibody, conjugated to a microtubule disrupting agent— monomethyl auristatin—via a stable, protease resistant linker
Belantamab mafodotin-blmf
Bone-targeting medication preferred in patients with renal insufficiency
Denosumab
Bone-targeting treatment (bisphosphonates or denosumab) should be continued for up to ______ years.
2 years
The frequency of dosing (monthly vs. every 3 months) would depend on the individual patient criteria, response to therapy, and agent used.
Continuing beyond 2 years should be based on clinical judgment.
Consider _________________ for symptomatic vertebral compression fractures.
Vertebroplasty or kyphoplasty
Orthopedic consultation should be sought for impending or actual long-bone fractures or bony compression of spinal cord or vertebral column instability.
Management of hypercalcemia
- Hydration
- Bisphosphonates (zoledronic acid preferred), denosumab
- Steroids and/or
- Calcitonin
Therapy for symptomatic hyperviscosity
Plasmapheresis
Factors associated with an increased risk of infections in patients with MM:
- Autologous HCT
- Bispecific antibody (BsAb) therapy
- CAR T-cell therapy
- Cytotoxic chemotherapy
- New diagnosis of MM has an increased risk of bacterial infection.
- Proteasome inhibitors and monoclonal antibodies have an increased risk of viral infection.
- High-dose steroids have an increased risk of fungal infections.
VTE Risk stratification scoring used in MM
- IMPEDE Score
- SAVED Score
IMPEDE Score
Individual Risk Factors
* Central venous catheter/Tunneled central line +2
* Pelvic, hip, or femur fracture +4
* Obesity (body mass index ≥25) +1
* Previous VTE +5
Myeloma Risk Factors
* Immunomodulatory drug (IMiD) +4
* Erythropoiesis-stimulating agent +1
* Dexamethasone <160 mg/month +2
* Dexamethasone >160 mg/month +4
* Doxorubicin or multiagent chemotherapy +3
Negative Factors
* Ethnicity/Race = Asian/Pacific Islander -3
* Existing thromboprophylaxis: prophylactic LMWH (low-molecular-weight heparin) or aspirin -3
* Existing thromboprophylaxis: therapeutic LMWH or warfarin -4
SAVED Score
- Surgery within 90 days +2
- Asian race -3
- VTE history +3
- Age ≥80 years +1
- Dexamethasone (regimen dose)
- Standard dose (120–160 mg/cycle) +1
- High dose (>160 mg/cycle) +2
The highest risk for VTE is in the
First 6 months following new diagnosis of MM
VTE Prophylaxis Recommendations
≤3 Points by IMPEDE Score or <2 Points by SAVED Score
Aspirin 81–325 mg once daily
VTE Prophylaxis Recommendations
≥4 Points by IMPEDE Score or ≥2 SAVED Score
- LMWH (equivalent to enoxaparin 40 mg daily) OR
- Rivaroxaban 10 mg daily OR
- Apixaban 2.5 mg twice daily OR
- Fondaparinux 2.5 mg daily OR
- Warfarin (target INR 2.0–3.0)
A less common choice of agent includes dalteparin 5,000 units subcutaneously (SC) daily
Duration of VTE Prophylaxis
- Indefinite while on myeloma therapy
- 3–6 months followed by aspirin (longer periods of anticoagulation may be considered in the presence of additional patient, treatmentspecific, or transient VTE risk factors)
Treatment Options for Renal Disease in Myeloma
Defined as serum creatinine >2 mg/dL or established glomerular filtration rate (eGFR) < 60 mL/min/1.73 sqm.
- Pulse dexamethasone
- Regimens containing bortezomib and/or daratumumab
Can switch to other regimen once renal function has improved or stabilized
Lenalidomide Dosing in Patients with Multiple Myeloma Who Have Renal Impairment
- CLCr ≥30 mL/min to <60 mL/min
- CLCr <30 mL/min (not requiring dialysis)
- CLCr <30 mL/min (requiring dialysis)
- CLCr ≥30 mL/min to <60 mL/min: 10 mg every 24 h
- CLCr <30 mL/min (not requiring dialysis): 15 mg every 48 h
- CLCr <30 mL/min (requiring dialysis): 5 mg once daily; on dialysis days, dose should be administered after dialysis
MONOCLONAL GAMMOPATHY OF RENAL SIGNIFICANCE
Renal biopsy recommended if:
- Acute kidney injury (AKI) stage 3
- eGFR <60 mL/min and >2 mL/min per year decline
- Proteinuria (>1 g/day)
- Albumin:creatinine >30 mg/mmol
- Fanconi syndrome
The biopsy may be deferred if the eGFR is stable, the urinalysis is normal, or there is no evidence of proteinuria (it is not always light chain proteinuria).
MONOCLONAL GAMMOPATHY OF NEUROLOGICAL SIGNIFICANCE
Rule out other causes of neuropathy
- Diabetes
- Cobalamin deficiency
- Thyroid dysfunction
- Lyme disease
- HIV infection
- Syphilis
- Autoimmune disease
- Cryoglobulinemia
- Evaluation for light chain amyloidosis,, WM or POEMS (POEMS-1), if appropriate
MONOCLONAL GAMMOPATHY OF NEUROLOGICAL SIGNIFICANCE
Clinical Findings High suspicion for IgM MGNS (Monoclonal gammopathy of neurological significance)
(+) Anti-MAG antibodies
- Sensory predominant
- Length dependent
- Slow progression (years)
- Bilateral and symmetrical
- Antibodies present
- Demyelination by EMG/NCS OR intermediate suspicion (not high or low suspicion) AND Affecting activities of daily living (ADLs)
Low suspicion
* Motor/pain predominant
* Non-length dependent
* Rapid progression (weeks to months)
* Unilateral/asymmetrical
* Antibodies not present
* No demyelination by EMG/NCS OR intermediate/high suspicion AND not affecting ADLs
POEMS (POLYNEUROPATHY, ORGANOMEGALY, ENDOCRINOPATHY, MONOCLONAL PROTEIN,
Criteria for the Diagnosis of POEMS Syndrome
Both of the mandatory major criteria, one of the other three major criteria, and one of the six minor criteria are present
Mandatory major criteria
* Polyneuropathy (typical demyelinating)
* Monoclonal plasma cell-proliferative disorder (almost always λ)
Other major criteria (one required)
* Castleman disease
* Sclerotic bone lesions
* Vascular endothelial growth factor elevation
Minor criteria
* Organomegaly (splenomegaly, hepatomegaly, lymphadenopathy)
* Extravascular volume overload (edema, pleural effusion, or ascites)
* Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic)
* Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, white nails)
* Papilledema
* Thrombocytosis/polycythemia
Other signs and symptoms:
Clubbing, weight loss, hyperhidrosis, pulmonary hypertension, restrictive lung disease, thrombotic diatheses, diarrhea, low vitamin B12 levels
POEMS is believed to be correlated with inflammatory cytokines, such as
Vascular endothelial growth factor (VEGF)
POEMS
Treatment for isolated bone lesion (<3 sites) in patients without clonal BMPC
RT
POEMS
Induction therapy options for transplant-eligible include:
- Lenalidomide/dexamethasone
- Bortezomiba/dexamethasone
- Melphalan/dexamethasone
- Cyclophosphamide/dexamethasone
- Pomalidomide/dexamethasone
POEMS
Induction therapy options for transplant-ineligible include:
- Lenalidomide/dexamethasone
- Bortezomiba/dexamethasone
- Melphalan/dexamethasone
- Cyclophosphamide/dexamethasone
- Pomalidomide/dexamethasone
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