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Blood, 1 October 2002, Vol. 100, No. 7, pp. 2679-2680
CORRESPONDENCE
To the editor:
Long-term follow-up on a patient with incomplete POEMS syndrome
undergoing high-dose therapy and autologous blood stem cell
transplantation
Jaccard et al recently reported a series of 5 patients with
polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome who underwent high-dose
chemotherapy followed by autologous stem cell
transplantation.1 In 2001, first reports of single
patients after autologous transplantations were
published.2,3 So far, all published cases show dramatic improvement in clinical manifestations, especially of polyneuropathy, and no relapses have been reported after autologous stem cell transplantation. However, data on long-term follow-up are extremely rare. Median follow-up of patients published by Jaccard et al was 36 months, with the first patient being assessed 58 months after transplantation. Here, we report a patient who developed multifocal osteosclerotic
myeloma in 1994 at the age of 30. Osteosclerosis was diagnosed in the
skull, both femura, and the pelvis, and the diagnosis was confirmed by
biopsy. Bone marrow histology revealed no increase in the
number of plasma cells. Due to progressive polyneuropathy predominating
the lower limbs, the patient rapidly became paraplegic. Muscular
strength of the arms was considerably reduced as well. All muscular
stretch reflexes were absent. There was hypesthesia and
pallhypesthesia of the lower extremities. Electroneurographic studies revealed that nerves of the lower extremities had no
motorial or sensoric nerve action potentials, and F waves were
absent. Also, nerves of the upper extremities showed
electroneurographic abnormalities with reduction of nerve conduction
velocity and nerve amplitudes. Incomplete POEMS syndrome of
polyneuropathy, hepatomegaly and lymphadenopathy, peripheral edema,
thrombocytosis (700 000/µm), and pleural effusion was diagnosed
according to Gheradi et al.4 In contrast to other
published cases, no monoclonal immunoglobulin was detected
(immunofixation electrophoresis of serum and urine, on follow-up free
light chains by nephelometry). Although therapy was started (melphalan, prednisolone), neurological
symptoms progressed further. After ineffective tamoxifen medication,5 the patient received 4 cycles of ifosfamide
(2 × 3000 mg/m2). Peripheral blood stem cells were
collected and the patient underwent high-dose chemotherapy with total
body irradiation (12 Gy) and melphalan (140 mg/m2) in April 1996. The posttransplantation course was
uneventful except for neutropenic fever and severe mucositis. Six years
after transplantation the patient is still in good clinical condition. His neurologic status improved considerably; the patient is able to get
up and walk short distances on crutches including going upstairs a few steps. Extensor and flexor muscles of the feet were
still plegic. All other muscles of the upper and lower extremities had
nearly normal strength. Hypesthesia and pallhypesthesia of the
lower extremities had slightly improved. All muscular stretch reflexes
were absent. Electroneurographic parameters of nerves of the upper
extremities normalized. Some of the sclerotic lesions improved (femura,
pelvis), some remained unchanged (skull), and no new osteosclerotic
lesions occurred. Thrombocyte counts returned to normal, and peripheral
edema disappeared. Observed clinical and laboratory improvements could
also have at least partially resulted from the mobilization
chemotherapy. After 6 years of follow-up, there are no clinical or
biochemical signs of progression. This report demonstrates that, as shown by Jaccard et al,1
autologous stem cell transplantation should be considered in patients
with POEMS syndrome. This patient showed a good partial remission now
lasting more than 6 years after transplantation.
Anne Wiesmann, Robert Weissert, Lothar Kanz, and Hermann Einsele
Correspondence: Hermann Einsele, Department of Hematology,
Oncology, Immunology and Rheumathology, Division of Medicine,
University of Tübingen, Otfried-Müller Str. 10, 72076 Tübingen, Germany; e-mail: hermann.einsele{at}med.uni-tuebingen.de.
References
1.
Jaccard A, Royer B, Bordessoule D, Brouet JC, Fermand JP.
High-dose therapy and autologous blood stem cell transplantation in POEMS syndrome.
Blood.
2002;99:3057-3059[Abstract/Free Full Text].
2.
Hogan WJ, Lacy MQ, Wiseman GA, Fealey RD, Dispenzieri A, Gertz MA.
Successful treatment of POEMS syndrome with autologous hematopoietic progenitor cell transplantation.
Bone Marrow Transplant.
2001;28:305-309[CrossRef][Medline]
[Order article via Infotrieve].
3.
Rovira M, Carreras E, Blade J, et al.
Dramatic improvement of POEMS syndrome following autologous haematopoietic cell transplantation.
Br J Haematol.
2001;115:373-375[CrossRef][Medline]
[Order article via Infotrieve].
4.
Gherardi RK, Belec L, Soubrier M, et al.
Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome.
Blood.
1996;87:1458-1465[Abstract/Free Full Text].
5.
Enevoldson TP, Harding AE.
Improvement in the POEMS syndrome after administration of tamoxifen.
J Neurol Neurosurg Psychiatry.
1992;55:71-72[Free Full Text].
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