a74

Correspondence

Castleman Disease in POEMS Syndrome with Elevated Interleukin-6

Recently, serum interleukin-6 (IL-6) levels were reported to be elevated in POEMS syndrome (polyneuropafhy, organo- megaly, endocrinopathy, monoclonal protein, and skin le- sions) associated with Castleman disease (CD).’ Elevated serum IL-6 levels have also been reported in CD.2

In a systematic survey, we studied 80 patients with previ- ously untreated multiple myeloma (MM), 1 patient with ab- dominal angiofollicular CD, and 9 patients with POEMS syn- drome. We used a specific and sensitive immunoenzymatic assay (Medgenix EASIA IL-6)3 to evaluate serum IL-6 levels. The nine patients with POEMS syndrome‘ had chronic pe- ripheral neuropathy and some clinical features of the POEMS syndrome. They all presented a plasmocytic proliferation as- sociated with a serum monoclonal immunoglobulin (IgCX: 5 patients; IgAX: 3 patients; and IgDX: 1 ~ a t i e n t ) . ~ None of these cases was associated with CD.

Normal IL-6 levels are below 15 pg/ml in healthy donors. Elevated serum IL-6 levels were detected in 43% of the pa- tients in the MM group (IL-6 values ranged from 16 to 2700 pg/ml), and in the one patient with CD (IL-6 > 2000 pg/ml). IL-6 levels were undetectable in all of the cases of POEMS syndrome (< 15 pg/ml).

Mandler and associates suggest that 1L-6 may be one factor contributing to manifestations of POEMS syndrome. How- ever, other authors have suggested that the increased produc- tion of IL-6 plays a major role in the development of systemic symptoms of CD.2,5 Our findings are consistent with these data. Therefore, we propose that IL-6 hyperproduction is probably related more to CD than to POEMS syndrome.

References

Mandler RN, Kerrigan DI’, Smart J , Kuis W, Villiger 1’, Lotz M. Castleman’s disease in POEMS syndrome with elevated inter- leukin-6. Cflrrc.tvr 1992; 69:2697-703. Yoshi7.aki K, Matsuda T, Nishimoto N, Kuritani T, Taeho L, Ao- zasa K, et al. Pathogenic significance of interleukin-6 (IL-O/BSF- 2) in Castleman’s disease. 1JIood 1989; 74: 1360-7. Crau GE. Implicationsof cytokines in immunopathology: experi- mental and clinical data. Eur C!/kiliIrit- Nt71ui 1990; 1:203-10. l)elauchc-Cavallier MC, Clauvel JC, Danon F, Peraldi MN, Di- vine M, Seligmann M. Keuropathie pbriphkrique et pro- lifbrations plasmocytaires: a propos d e 17 observations. Arrrr Mrd Irrf iwt . (PnrIs) 1990; 143:651-6. Leger-Ravet MB, Peuchmaur M, Devergne 0, Audoin J, Raphael M, Van Danime J, et al. Interleukin-6 gene expression in Castle- man’s disease. Blood 1991; 78:2923-30.

Carole Einile Franqoise Danon, M.D. Jean-Paul Fermand, M.D. Jean-I’ierre Clauvel, M.D. Laboratory of Immunology and

Department of Clinical Immunology Hbpital Saint-Louis Paris, France

R e p l y to Emile et a l .

We appreciate the interest of Emile and associates in our paper.’ Unfortunately, Emile and associates d o not state the sensitivity of their interleukin-6 (IL-6) ELISA assay. I t is possi- ble that they have missed low levels of IL-6 that can be de- tected with the bioassay we used, which is probably more sensitive. Furthermore, it is likely that serum IL-6 levels will fluctuate with disease activity and severity (as demonstrated in our paper). Moreover, they present n o data about cerebro- spinal fluid IL-6. I n the absence of data on these clinical vari- ables, it is difficult to interpret the findings of Eniile and asso- ciates.

Reference 1. Mandler RN. Kerrigan Ill’, Smart J, Kuis W. Villiger P, Imtz M.

Castleman’s disease in I’OEMS syndrome with elevated IL-6. Cnricrr 1991; h9:2697-703.

R. N. Mandler D. P. Kerrigan J . Smart W. Kuis P. Villiger M. Lotz Department of Neurology School of Medicine The University of New Mexico Albuquerque, New Mexico

Mental Neuropathy (Numb Chin Syndrome): A Harbinger of Tumor Progression or Relapse

In response to the recent report of Burt et al.,’ we report two instances of lymphoma presenting with unilateral mental neu- ropathy.

Case Reports

Case I

A 47-year-old woman experienced right cervical adenopathy and frequent sweats. Stage 11B Hodgkin lymphoma was diag- nosed and complete remission was achieved after mantle radi- ation therapy. Eight months later, asymptomatic left axillary adenopathy developed. The patient was treated with six cy- cles of mechlorethamine, vincristine, procarbazine, and pred- nisone (MOPP) therapy, which led to a second complete re- mission.