Resident Rounds Part III: Plasma Cell Leukemia With Initial Cutaneous Presentation

August 2014 | Volume 13 | Issue 8 | Features | 994 | Copyright © August 2014


Stephanie K. Fabbro MD and Benjamin H. Kaffenberger MD

Division of Dermatology, Ohio State University Wexner Medical Center, Columbus, OH

medullary sites.4 Other sites that have been described as being more commonly involved include the subcutis, pleura, central nervous system and testes; the latter two possibly due to a leukemic sanctuary mechanism during treatment.5
Treatment options are not optimized, but therapeutic success defined as improving survival and quality of life has been described. Initial cases were treated using melphalan and prednisone; soon after, vinchristine, Adriamycin (doxorubicin), dexamethasone (VAD) was described in a number of cases as being therapeutically useful, increasing survival to 15-18 months.6 More recently, the proteasome inhibitor bortezomib, after successful studies in the treatment of MM, has shown promise, with a mean survival of 12.6 months after treatment. Stem cell transplantation has also been shown to be successful in a number of case report series and retrospective analyses with mean survival rates ranging from 34-45 months.7

CONCLUSION

PCL is an aggressive variant of MM, that rarely presents in the skin but when present portends a poor prognosis. Plasma cell dyscrasias should be considered in the differential of cases that clinically present violaceous, infiltrated deep set papules and nodules. Referral to a hematologist and potential clinical trials is warranted.

Disclosures

None of the authors have declared any relevant conflicts.

REFERENCES

  1. Chattopadhyay A, Nath UK, De R et al. Primary plasma cell leukemia with initial cutaneous involvement and IgA biclonal gammopathy. Ann Hematol 2008;87(3):249-51
  2. Alexandrescu DT, Koulova L, Wiernik PH. Unusual cutaneous involvement during plasma cell leukemia phase of multiple myeloma patient after treatment with thalidomide: a case report and review of the literature. Clin Exp Dermatol 2005;30:391-394.
  3. Cho-Vega JH, Medeiros J, Prieto VG, Vega F. Leukemia cutis. Anatom Path 2008; 129:130-142.
  4. Tsutani H, Sugiyama T, Shimizu S, et al. Discordant LFA-1/ICAM-1 expression in a case of secondary plasma cell leukemia associated with subcutaneous plasmocytoma. Am J Hematol 1993;42:299-304.
  5. Bladé J, Kyle RA. Nonsecretory myeloma, immunoglobulin D myeloma, and plasma cell leukemia. Hematol Oncol Clin 1999;13:1259–1272.
  6. Costello R, Sainty D, Bouabdallah R et al. Primary plasma cell leukaemia: a report of 18 cases. Leuk Res 2001;25:103–107
  7. Garcia-Sanz R, Orfao A, Gonzalez M et al. Primary plasma cell leukemia: clinical, immunophenotypic, DNA ploidy, and cytogenetic characteristics. Blood 1999;93:1032.

AUTHOR CORRESPONDENCE

Stephanie K. Fabbro MDstephanie.fabbro@osumc.edu
Close Menu