Makindo Medical Notes

🧾 About POEMS Syndrome

• Polyneuropathy: Typically presents as a progressive, symmetrical, demyelinating neuropathy resembling CIDP 🧠. Patients develop distal weakness, sensory loss, and areflexia.
• Organomegaly: Most often hepatomegaly, but splenomegaly and lymphadenopathy may also be present. ⚠️ These features help distinguish POEMS from isolated plasma cell disorders.
• Endocrinopathy: Common abnormalities include hypogonadism (erectile dysfunction, infertility, amenorrhoea), gynaecomastia, hypothyroidism, adrenal insufficiency, and type 2 diabetes mellitus 🍬.
• Monoclonal Plasma Cell Disorder: Usually a lambda light chain monoclonal gammopathy, detectable on serum electrophoresis. Bone marrow biopsy shows <5% clonal plasma cells.
• Skin Changes: Hyperpigmentation, sclerodermoid changes, hypertrichosis, and angiomas (cherry haemangiomas 🍒 are classic).

📌 Mnemonic: POEMS = Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes.

🧪 Investigations

• Blood Tests:
  • Raised ESR 📈
  • Fasting glucose or HbA1c may show diabetes
  • Endocrine screen: thyroid, cortisol, testosterone/oestrogen, prolactin
• Serum Protein Electrophoresis (SPEP/UPEP): Detects monoclonal (M) band — typically IgA or IgG lambda.
• VEGF levels: Markedly elevated in most patients — useful as a diagnostic marker and for monitoring treatment response 🧬.
• Cytokines: Raised IL-6, TNF-α ➝ drive systemic features (fever, weight loss, inflammation).
• Imaging:
  • USS/CT ➝ hepatosplenomegaly, lymphadenopathy
  • Skeletal survey or MRI ➝ osteosclerotic bone lesions (seen in ~50% cases, unlike lytic lesions of myeloma).
• Nerve conduction studies: Show demyelination and conduction block.

⚖️ Differential Diagnosis

• Multiple myeloma (lytic lesions, higher marrow plasma cell burden)
• Castleman’s disease (may coexist with POEMS)
• CIDP (but lacks systemic/endocrine features)
• Other causes of neuropathy (diabetes, alcohol, B12 deficiency)

💊 Management

• Targeted therapy:
  • Localised disease: Radiotherapy to solitary plasmacytomas.
  • Systemic disease: Chemotherapy (melphalan, corticosteroids, lenalidomide, bortezomib).
  • Autologous Stem Cell Transplant (ASCT): Best long-term outcomes in eligible patients 🌱.
• Supportive management: Endocrine replacement (thyroxine, testosterone, insulin), physiotherapy for neuropathy, pain management.
• Prognosis: 5-year survival ~60–70%. Better if diagnosed early before severe neuropathy or multi-organ dysfunction.

🚨 Exam Tip: Look for a patient with neuropathy + endocrinopathy + skin changes + organomegaly ➝ think POEMS, not CIDP or myeloma.

Cases — POEMS Syndrome

• Case 1 — Progressive Neuropathy with Endocrine Signs: A 55-year-old man presents with gradually worsening tingling and weakness in his feet, now requiring a stick to walk. Exam: symmetrical sensorimotor polyneuropathy. He has hyperpigmented skin, hepatosplenomegaly, and gynaecomastia. Bloods: monoclonal IgA lambda paraprotein. Diagnosis: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes). Management: Treat underlying plasma cell disorder (e.g. radiotherapy for isolated plasmacytoma, systemic therapy for widespread disease), supportive neuropathy care, endocrinology input.
• Case 2 — Skin Changes and Papilloedema: A 48-year-old woman presents with fatigue, skin thickening, and increased hair growth. She reports worsening vision. Exam: hyperpigmentation, white nails, and papilloedema on fundoscopy. Nerve conduction studies: demyelinating neuropathy. Serum electrophoresis shows monoclonal IgG lambda protein. Diagnosis: POEMS syndrome with papilloedema. Management: Haematology referral; systemic therapy (lenalidomide + dexamethasone or autologous stem cell transplant if eligible); manage endocrinopathies; visual monitoring.

Teaching Commentary 🧾

POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell dyscrasia (often osteosclerotic myeloma). Key features (mnemonic POEMS): - Polyneuropathy (demyelinating, progressive, disabling), - Organomegaly (hepatosplenomegaly, lymphadenopathy), - Endocrinopathy (hypogonadism, hypothyroidism, diabetes), - Monoclonal plasma cell disorder (IgA/IgG, lambda restricted), - Skin changes (hyperpigmentation, hypertrichosis, white nails, thickening). Other: papilloedema, sclerotic bone lesions, extravascular fluid overload. Management focuses on controlling the plasma cell clone (radiotherapy if localised, systemic therapy or transplant if disseminated) with multidisciplinary supportive care.