Poems Syndrome with Vitiligo: Complete Remission and Repigmentation Post Autologous Stem Cell Transplant

Case Report

Ann Hematol Oncol. 2017; 4(8): 1165.

Poems Syndrome with Vitiligo: Complete Remission and Repigmentation Post Autologous Stem Cell Transplant

Raheel Iftikhar*, Syed Kamran Mahmood, Tariq Mehmood Satti and Qamar un nisa Chaudhry

Armed Forces Bone Marrow Transplant Centre, Pakistan

*Corresponding author: Raheel Iftikhar, Armed Forces Bone Marrow Transplant Centre, Rawalpindi, Pakistan

Received: June 12, 2017; Accepted: July 03, 2017; Published: July 24, 2017


The acronym POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) represents a rare paraneoplastic syndrome due to underlying plasma cell disorder. Due to myriad non-specific presentation, most of these cases are missed or diagnosis is significantly delayed. The importance of early correct diagnosis is enormous as POEMS syndrome carries a significantly better prognosis than most of the other plasma cell disorders. Vitiligo is not a known skin manifestation of POEMS syndrome and often cosmetically severely disfiguring and very difficult to treat.

We are reporting case of a middle age male presenting with POEMS syndrome and vitiligo who underwent autologous stem cell transplant and achieved complete remission of POEMS syndrome and had skin repigmentation post hematopoietic stem cell transplant (HSCT).

Keywords: Polyneuropathy, Monoclonal gammopathy, Vitiligo, Stem cell transplantation


POEMS syndrome also known as osteosclerotic myeloma, Crowfukase syndrome or Takatsuki syndrome is a rare monoclonal plasma cell disorder. Most of the clinical manifestations of this syndrome are remembered by acronym “POEMS” although it inadequately covers many defining features of this myriad syndrome [1]. Due to rarity of the syndrome and multisystem involvement, diagnosis is often delayed. Exact pathogenesis is not well known and is believed to be due to chronic over production of pro-inflammatory cytokines particularly vascular endothelial growth factor (VEGF). Risk adapted therapy, use of proteasome inhibitors and HSCT has resulted in dramatic improvement in long term survival of patients suffering from this disease [2]. Although patients with POEMS syndrome have many skin manifestations, vitiligo is not a documented cutaneous manifestation. We hereby present a case of POEMS syndrome and vitiligo in which disease remission post HSCT was also associated with regimentation of skin and resolution of vitiligo. To our knowledge, this is first case report highlighting this aspect in POEMS syndrome.

Case Presentation

A 46 years old male presented in outpatient department with 2 years history of off and on numbness of hands and feet, facial twitching and abdominal pain. With these complaints he was reviewed by endocrinologist and was found to have primary hypoparathyroidism and was started on calcium and vitamin-D replacement. He was investigated and no cause of hypoparathyroidism could be found. Two months late he had an episode of severe abdominal pain and was operated for appendicitis but histopathology of tissue showed normal non-inflamed appendix. His abdominal pain persisted and he started having low backache and numbness of hands and feet which were attributed initially to hypoparathyroidism, however numbness of hands and feet progressed and he developed glove and stocking peripheral neuropathy. He also noted appearance of hypopigmented skin patches over face and arms which progressed to involve trunk and back and was associated with excessive hair growth all over body. He was reviewed by dermatologist and advised narrow band ultraviolet B therapy and topical hydroquinone for vitiligo, however there was no improvement. His back pain worsened gradually and he also started having pain in left hip joint with antalgic gait. His blood complete picture done as a part of routine screening showed raised hemoglobin and platelet count and was referred to our institute for evaluation. On examination he had vitiligo, hyperpigmentation, hypertrichosis and white nails. Systemic examination revealed hepatosplenomegaly and glove and stocking peripheral neuropathy. Lab investigations revealed hemoglobin of 16.7 g/dl, hematocrit 49%, platelet counts of 560,000/ul. Bone marrow aspiration and trephine biopsy showed 6% plasma cells which were CD38 and CD138 positive with increased megakaryocytes. PCR for JAK-2 mutation was negative. Serum protein electrophoresis showed a faint band in gamma region. His serum free light chains showed increase in lambda light chain with kappa: lambda of 4.2 (normal 0.6-1.65) and serum immunofixation showed IgG lambda. His Contrast enhanced computed tomography scans showed hepatosplenomegaly, lytic lesion left femur and mixed sclerotic and lytic lesions involving dorsolumbar spine (Figure 1A). Biopsy of vertebral sclerotic lesions was done and was suggestive of plasmacytoma. EMG/NCS showed sensorimotor distal axonal neuropathy involving all 4 limbs. His endocrine profile was repeated which showed primary hypoparathyroidism and hypogonadotrophic hypogonadism. His PET scan showed hypermetabolic skeletal, pulmonary and adrenal signals (Figure 1B). During admission he developed an episode of slurring of speech and generalized tonic clonic seizure. MRI brain was done, which showed acute parietooccipital infarct.