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Q1. 下列對於 POEMS syndrome 之 IMWG diagnosis criteria，何者正確？

(1) Polyneuropathy。 (2) Monoclonal plasma cell proliferative disorder，通常為 kappa。 (3) Organomegaly，endocrinopathy，skin changes 均為 minor criteria 之一。 (4) Anemia and/or thrombocytopenia 為 3 個 major criteria 之一。

  • (A) 1,2,3 正確
  • (B) 1,3 正確
  • (C) 2,4 正確
  • (D) 4 正確
  • (E) 以上皆正確
點此顯示正解

(B) 1,3 正確

詳解

The correct answer is (B) "1,3 correct" based on the IMWG diagnostic criteria for POEMS syndrome.

POEMS-3: Multiple Myeloma: Criteria for the Diagnosis of POEMS Syndrome — NCCN Guidelines® — Multiple Myeloma p. 46 (v5.2026)

Why Answer (B) is Correct

Statement (1) is CORRECT: Polyneuropathy (typically demyelinating) is one of the two mandatory major criteria required for POEMS syndrome diagnosis1235.

Statement (3) is CORRECT: Organomegaly, endocrinopathy, and skin changes are each individual minor criteria in the IMWG classification. The six minor criteria include: (6) organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy), (7) extravascular volume overload, (8) endocrinopathy, (9) skin changes, (10) papilledema, and (11) thrombocytosis/polycythemia1235.

Why Statements (2) and (4) are Wrong

Statement (2) is INCORRECT: The monoclonal plasma cell proliferative disorder in POEMS syndrome is almost always lambda (λ), NOT kappa. This is explicitly stated in the IMWG criteria as "almost always λ"1235. The lambda subtype is found in >95% of cases6, with highly restricted usage of specific lambda variable domains (IGLV1-40 and IGLV1-44)7[^9]. Among patients with detectable monotypic plasma cells, 91% express lambda light chain[^10].

Statement (4) is INCORRECT: Anemia and/or thrombocytopenia are NOT among the major criteria for POEMS syndrome. In fact, the NCCN guidelines explicitly note that "anemia and/or thrombocytopenia are distinctively unusual in this syndrome unless Castleman disease is present"1. The actual hematologic abnormality listed as a minor criterion is thrombocytosis/polycythemia (criterion 11), which is the opposite of thrombocytopenia1235. The presence of anemia or thrombocytopenia should raise suspicion for coexistent Castleman disease rather than being a diagnostic feature of POEMS syndrome itself.

The IMWG diagnostic criteria require: both mandatory major criteria (polyneuropathy + monoclonal plasma cell disorder) + one of three other major criteria (Castleman disease, sclerotic bone lesions, or elevated VEGF) + one of six minor criteria1235.

詳解 · 中文翻譯

根據 POEMS 症候群的 IMWG 診斷標準，正確答案是 (B)「1,3 正確」。

為何答案 (B) 正確

陳述 (1) 正確：多神經病（通常為脫髓鞘性）是 POEMS 症候群診斷所需的 兩個強制主要標準之一1235。

陳述 (3) 正確：器官腫大、內分泌病和皮膚改變在 IMWG 分類中各自為單獨的次要標準。六個次要標準包括：(6) 器官腫大（脾腫大、肝腫大或淋巴結腫大）、(7) 血管外容量超負荷、(8) 內分泌病、(9) 皮膚改變、(10) 視乳頭水腫和 (11) 血小板增多症/紅細胞增多症1235。

為何陳述 (2) 和 (4) 錯誤

陳述 (2) 不正確：POEMS 症候群中的單克隆漿細胞增殖性疾病幾乎總是 lambda（λ），而非 kappa。這在 IMWG 標準中明確指出為「幾乎總是 λ」1235。Lambda 亞型見於 >95% 的病例6，高度限制特定 lambda 可變結構域的使用（IGLV1-40 和 IGLV1-44）7[^9]。在可檢測到單型漿細胞的患者中，91% 表達 lambda 輕鏈[^10]。

陳述 (4) 不正確：貧血和/或血小板減少症不在 POEMS 症候群的主要標準中。實際上，NCCN 指南明確指出「貧血和/或血小板減少症在此症候群中明顯不尋常，除非存在 Castleman 病」1。列為 次要標準的實際血液學異常是 血小板增多症/紅細胞增多症（標準 11），這與血小板減少症相反1235。貧血或血小板減少症的存在應引起對共存 Castleman 病的懷疑，而非 POEMS 症候群本身的診斷特徵。

IMWG 診斷標準要求：兩個強制主要標準（多神經病 + 單克隆漿細胞疾病）+ 三個其他主要標準之一（Castleman 病、硬化性骨損害或升高 VEGF）+ 六個次要標準之一1235。

參考文獻 (AMA)

  1. National Comprehensive Cancer Network. Multiple Myeloma. https://www.nccn.org/professionals/physician_gls/pdf/myeloma.pdf#page=46. ↩↩↩↩↩↩↩↩↩↩↩↩

  2. Dispenzieri A. POEMS Syndrome: 2021 Update on Diagnosis, Risk-Stratification, and Management. American Journal of Hematology. 2021;96(7):872-888. doi:10.1002/ajh.26240. PMID:34000085. ↩↩↩↩↩↩↩↩↩↩

  3. Dispenzieri A. POEMS Syndrome: Update on Diagnosis, Risk-Stratification, and Management. American Journal of Hematology. 2023;98(12):1934-1950. doi:10.1002/ajh.27081. PMID:37732822. ↩↩↩↩↩↩↩↩↩↩

  4. Dispenzieri A. POEMS Syndrome: 2019 Update on Diagnosis, Risk-Stratification, and Management. American Journal of Hematology. 2019;94(7):812-827. doi:10.1002/ajh.25495. PMID:31012139. ↩

  5. Khouri J, Nakashima M, Wong S. Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review. JAMA Oncology. 2021;7(9):1383-1391. doi:10.1001/jamaoncol.2021.0586. PMID:34081097. ↩↩↩↩↩↩↩↩↩↩

  6. Bender S, Javaugue V, Saintamand A, et al. Immunoglobulin Variable Domain High-Throughput Sequencing Reveals Specific Novel Mutational Patterns in POEMS Syndrome. Blood. 2020;135(20):1750-1758. doi:10.1182/blood.2019004197. PMID:32243509. ↩↩

  7. Dao LN, Hanson CA, Dispenzieri A, et al. Bone Marrow Histopathology in POEMS Syndrome: A Distinctive Combination of Plasma Cell, Lymphoid, and Myeloid Findings in 87 Patients. Blood. 2011;117(24):6438-44. doi:10.1182/blood-2010-11-316935. PMID:21385854. ↩↩

Slide annotations

Anemia and/or thrombocytopenia are distinct unusual (unless Castleman).

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