POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes) is a rare paraneoplastic syndrome with multisystem involvement [1]. It typically arises from monoclonal plasma cell proliferation and is considered an atypical variant of multiple myeloma [2]. In the pathogenesis of the disease, markedly elevated levels of vascular endothelial growth factor (VEGF) play a crucial role, and most of the symptoms are associated with this mechanism [3].

The clinical presentation of POEMS syndrome is quite heterogeneous. In most patients, the polyneuropathy is a subacute, distal, sensorimotor and progressive demyelinating neuropathy; motor involvement is often prominent and significantly impairs patients' quality of life [4]. Organomegaly particularly hepatomegaly, splenomegaly, and lymphadenopathy is commonly observed. Endocrinopathy may present with a wide spectrum of disorders, including diabetes mellitus, hypothyroidism, and hypogonadism. Monoclonal gammopathy frequently of the λ (lambda) light chain type is a critical diagnostic finding. Cutaneous manifestations may include hyperpigmentation, hemangiomas, excessive hair growth (hypertrichosis), and skin thickening. Additional features can include papilledema, edema, ascites, pulmonary hypertension, and thromboembolic events [5,6].

The diagnostic criteria were first defined by Dispenzieri and colleagues and are currently based on a system of ‘major and minor criteria.’ For diagnosis, in addition to the two mandatory major criteria (polyneuropathy and monoclonal plasma cell proliferation), at least one additional major criterion and one minor criterion must be present. Measurement of VEGF levels is an important biomarker both for diagnosis and for monitoring treatment response [5].

Treatment is aimed at eliminating the underlying clonal plasma cell population. In patients with localized bone lesions, radiotherapy may be effective particularly in cases of limited disease. For widespread disease, systemic therapies are preferred. Immunomodulatory agents such as lenalidomide and thalidomide, as well as bortezomib based regimens, have been found effective. Autologous hematopoietic stem cell transplantation (HSCT) can provide long-term remission in suitable patients. Monitoring treatment response via VEGF levels shows that reductions in VEGF parallel clinical improvement [7,8].

Prognosis has markedly improved with treatment. Contemporary approaches have increased the 5-year survival rate to approximately 60–70%. However, delayed diagnosis—due to frequent misattribution of symptoms to other neurological or endocrine disorders—is a significant issue at presentation. Therefore, multidisciplinary collaboration among hematologists, neurologists, and endocrinologists is critical for timely diagnosis and effective treatment [9].

In conclusion, POEMS syndrome is a rare but clinically highly complex disorder. Early diagnosis and appropriate treatment improve both survival and quality of life. Given the syndrome’s clinical heterogeneity, increasing awareness especially within hematology practice is of great value [10].