POEMS 症候群

Buzzwords → Dx

Diagnostic Criteria (Mayo, IMWG-endorsed)

Mandatory (both):

1. Polyneuropathy (typically demyelinating).
2. Monoclonal plasma cell disorder (almost always λ).

Major (≥1 of 3):

• Castleman disease.
• Sclerotic bone lesion(s).
• VEGF elevation (≥4× upper normal).

Minor (≥1 of 6): organomegaly (spleen/liver/LN), extravascular volume overload (edema/effusion/ascites), endocrinopathy (excluding type 2 DM/hypothyroid alone), skin changes, papilledema, thrombocytosis/polycythemia.

Other features: weight loss, hyperhidrosis, pulmonary HTN, restrictive lung dz, diarrhea, low B12, thrombotic events.

Workup

• EMG + NCS confirm demyelinating polyneuropathy.
• SPE + IFE + sFLC + 24-hr urine.
• Marrow + skeletal survey + dedicated CT/MRI of sclerotic lesions.
• Serum VEGF.
• Endocrine panel (TSH, free T4, AM cortisol, testosterone, LH/FSH, A1c).
• Echo + PFTs (pulmonary HTN, restrictive disease).
• Excisional LN biopsy if Castleman suspected.

Treatment Algorithm

flowchart TD
  A[Confirmed POEMS] --> B{Disease extent}
  B -- localized 1-3 sclerotic lesions<br>no marrow --> C[Involved-field RT 40-50 Gy<br>often curative]
  B -- disseminated / marrow+ --> D{Auto-HCT eligible?}
  D -- yes --> E[Induction lenalidomide-dex × 4-6<br>or bortezomib<br>then auto-HCT]
  D -- no --> F[Lenalidomide-dex<br>or bortezomib-dex<br>continuous]
  E --> G[Long-term FU<br>VEGF + neuro improvement]
  F --> G
  G -- relapse --> H[Repeat induction or<br>switch class]

Pearls / Pitfalls

• Always λ light chain — if κ, reconsider diagnosis.
• Osteosclerotic (NOT osteolytic) lesions — the opposite of MM. Don't be confused by "plasma cell" overlap.
• VEGF is the single most useful biomarker — markedly elevated, drops with treatment response.
• Localized POEMS (1–3 sclerotic lesions, no marrow) can be CURED with radiation alone.
• Lenalidomide-dex or bortezomib-dex are first-line systemic options; auto-HCT is highly active for fit pts with disseminated disease (durable remissions).
• Steroids alone can transiently improve neuropathy but are NOT definitive — don't stop treatment there.
• Capillary leak / volume overload is dramatic — diuretics cautiously; treat underlying disease.
• Thrombosis risk is high (arterial + venous) — primary prophylaxis with aspirin during lenalidomide.
• Don't confuse with CIDP — CIDP responds to IVIG/steroids; POEMS does not respond well to IVIG.
• Castleman disease overlap: if multicentric Castleman is dominant, treat with siltuximab (anti-IL-6) ± rituximab.

Cross-references

• MM — osteolytic vs osteosclerotic contrast
• AL Amyloid — light-chain plasma-cell DDx
• Castleman / IgM disorders
• Drug Regimens — lenalidomide-dex, bortezomib

相關題目

• Q-067 — POEMS — clinical recognition
• Q-068 — POEMS — localized vs disseminated treatment
• Q-069 — POEMS — distinguishing kappa vs lambda

Sources