POEMS syndrome, osteosclerotic myeloma and Castleman’s disease: a case report J. Bosco Associate Professor, Department of Medicine University Hospital, University of Malaya, Kuala Lumpur, Malaysia
R. Pathmanathan Associate Professor, Department of Pathology University of Malaya, Kuala Lumpur, Malaysia
Abstract: Peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes are often the presenting features of the POEMS syndrome. Approximately 50% of these are associated with osteosclerotic myeloma, a rare variant of multiple myeloma and some with Castleman’s disease, an unusual lympho-proliferativedisorder. The underlying pathogenetic mechanisms have not been elucidated but amelioration of symptoms with the disappearance of the M-component suggests possible etiopathogenic mechanisms. We present a 40-year-old woman with manifestations of all three disorders. (Aust NZ J Med 1991; 21: 454-456.) Key words: POEMS syndrome, Castleman’s disease, osteosclerotic myeloma.
POEMS syndrome is an acronym for a unique multisystem disorder characterised by peripheral neuropathy, organomegaly, endocrine dysfunction, monoclonal gammopathy and skin changes.’ Crow described the first clinical case in 1956.’ The ‘Takatsuki syndrome’ and ‘CrowFukasa syndrome’ are other eponyms that are used for this unusual s y n d r ~ m e .Another ~,~ rare disorder that has been associated with this syndrome is Castleman’s disease: which classically is a localised form of mediastinal lymphoid hyperplasia characterised by hyalinisation of follicles and interfollicular vascular proliferation. Two subtypes have been recognised, a hyaline vascular type and a plasma cell variant.6 It is the plasma cell variant that is often associated with other systemic disorders. We report a female patient with all the features of POEMS and Castleman’s disease of th e hyalinevascular type. CASE REPORT A 40-year-old Indian woman presented to another hospital with a one year history of intermittent swelling ofupper and lower limbs, difficulty in walking and working with her hands, increased facial hair growth and darkening of her skin especially over the face, palms and soles. These changes had occurred gradually. On examination, she had excessive pigmentation, noticeable in spite of her ethnic origin, especially over the palms and soles. The skin was indurated, thickened and oedrrnatous. There was generalised lymphadenopathy Reprinz reyuesrs
and hepatosplenomegaly. A high-stepping gait with bilateral foot drop was noted. Neurological examination revealed extensive sensorimotor peripheral neuropathy of both upper and lower limbs. A clinical diagnosis of amyloidosis or lymphoma was made. A C T scan of the abdomen and pelvis confirmed the hepatosplenomegaly but in addition it showed extensive bony lesions involving the left ileum. Lymph node biopsy showed angiofollicular lymph node hyperplasia, Castleman’s disease, hyaline vascular type (Figure 1). As the clinical presentation did not entirely correlate with the known features of Castleman’s disease, she was referred to the University Hospital for definitive diagnosis and management. On direct questioning, the patient complained of amenorrhoea for about one year. There was no exposure to polyvinyl chlorides, other chemicals or drugs. There was no postural hypotension or papilloedema. Hypertrichosis of the face, arms and legs was noted. A clinical diagnosis of POEMS syndrome was made based on peripheral neuropathy, organomegaly, possible endocrinopathy, osteosclerotic lesions of ileum, hypertrichosis and hyperpigmentation of the skin. 1.aboratory investigations were as follows: Th e full blood count, electrolytes and renal function tests were normal. Th e total protein was 60 glL (67-85), serum albumin 30 glL (36-51) and alkaline phosphates ,350 IUlL (34-135). The other liver function tests were normal. Th e 24 hour urinary protein was 0.24 glL. Random blood sugar was 5.4 mmollL (3.8-6.1). Immunoglobulin quantitation: IgG 1150 mgldL (650-1500), IgA mgldL (75-312), IgM 195 mgldL (50-240). Agarose gel electrophoresis disclosed a M-component and was identified by immunofuration as IgG lambda protein. Serum electrophoresis was done and the
Associate Professor John Bosco, Department of Medicine, Faculty of Medicine, University of Malaya, 59100 Kuala Lumpur,
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BOSCO AND PATHMANATHAN
Figure 1: Section of lymph node (Haematoxylin rU Eosin, X 100) demonstrating angiofollicular lymph node hyperplasia. A prominent follicle is seen with vascular proliferation and hyalinisation of the centre. In the periphery of the follicle, concentric layer of lymphocytes results in an onion-skin appearance. Numerous hyperplastic vessels are present in the interfollicular stroma.
paraprotein quantitated at 4 giL. T h e test for antinuclear antibody was negative, C3:87 mgidL(83-177) and C4:16 mg/dL (15-45). No antithyroid or antiadrenal antibodies were found. Endocrinological abnormalities were confirmed as (i) primary hypothyroidism with increase in T S H 9.8 mIU/niL (