POEMS
Inhoudsopgave
Terug naar overzicht hematologische diagnosen http://de-heer.eu
1 verplicht aanvullend onderzoek
- algemeen bloedonderzoek
- M-proteine, evt. lichte ketens in serum/urine
- beenmergonderzoek
- biopt uit osteosclerotische lesie
- CT: osteosclerose, organomegalie, lymfadenopathie?
- i.c.c. neuro
- endocriene screening
- VEGF via UMCN
1.1 diagnostische criteria
- oorzaak onbekend
- verplichte criteria
- peripheral neuropathy
- predominantly motor chronic inflammatory demyelinating polyneuropathy
- autonomic symptoms are not a feature
- monoclonal plasma cell disorder, >90% lambda
- bij MGUS >90% <2 g/dL
- random bone marrow examination often nondiagnostic
- peripheral neuropathy
- major criteria, minimaal 1 vereist
- almost all patients : osteosclerotic lesions
- diffuse infiltration of light chain restricted plasma cells
- 66% elevated VEGF, geen gevalideerde cut-off, advies: >3-4x ULN
- 15% Castleman
- almost all patients : osteosclerotic lesions
- minor criteria, minimaal 1 vereist
- 50% Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)
- 29% Extravascular volume overload (peripheral edema, ascites, or pleural effusion)
- 67% Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, or pancreatic disorder excluding diabetes mellitus or hypothyroidism)
- 68% Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, white nails)
- Hyperpigmentation – 47 percent
- Hemangioma – 47 percent
- Hypertrichosis – 38 percent
- Acrocyanosis – 34 percent
- White nails – 30 percent
- Sclerodermoid changes – 26 percent
- Raynaud phenomenon – 20 percent
- Hyperemia/erythema – 20 percent
- Flushing – 16 percent
- Rubor – 11 percent
- Clubbing – 6 percent
- 29% Papilledema
- Thrombocytosis or polycythemia
- overig
- Increased cerebrospinal fluid protein (>50 mg/dL) – 100 percent
- Weight loss (>10 pounds) – 37 percent
- fatigue – 31 percent
2 let op! geen uiting of associatie met multipel myeloom!
- kan wel verwarring mee ontstaan door uitslag beenmerg/bot-onderzoek!
- none of our patients died of classic myeloma
- death due to renal failure: coexistent ascites and capillary leak-like syndrome without light chain deposition
3 therapie
- geen standaard therapie
- 1 tot max 3 osteosclerotische lesies: radiation in a dose of 40 to 50 Gy
- symptomatic and widespread osteosclerotic lesions or severe symptoms: systemic therapy similar to that employed in multiple myeloma
- MD (of LenDex hoewel minder ervaring mee) gevolgd door HDM bij fitte patienten
- Responsevaluatie: hematologic profile, imaging studies, VEGF levels, and symptom improvement: neuropathy typically 3 months to stabilize and 6 months to begin to improve, maximal improvement 2-3 years after definitive therapy
4 prognose
- chronic disease, median OS 13.7 years in the Mayo Clinic series
- progressive peripheral neuropathy until bedridden, death usually from uitputting or bronchopneumonia
- ongunstig: clubbing median survivals 2.6 years and extravascular volume overload had 6.6 years
- gunstig: patients who receive radiation therapy with a good response to treatment
- ongunstig m.b.t. progressie tot meer POEMS-symptomen: serum M-protein >1 g/dL, monoclonal light chains in urine, absence radiation therapy