B-cell chronic lymphocytic leukemia associated with high serum IGE levels and pruriginous skin lesions: successful therapy with IFN-alpha 2b after failure on IFN-gamma.
BACKGROUND
Chronic lymphocytic leukemia of the B-cell type (B-CLL) associated with highly elevated serum IgE levels and skin involvement has rarely been observed. Furthermore, not much is known about therapeutic strategies in such diseases.


OBJECTIVE
We describe a 56-year-old male patient with a 5-year history of chronic and relapsing pruritic skin lesions as well as recurrent Staphylococcus aureus infections of the skin.


RESULTS
Histologically, a lymphocytic and eosinophilic skin infiltrate was seen. Laboratory analysis revealed lymphocytosis (46.5%), eosinophilia (11.9%) as well as markedly elevated serum IgE levels (140,000 IU/ml). Immunohistology showed dermal B cells with intracytoplasmic IgE. Bone marrow biopsy showed a diffuse infiltrate of small lymphoplasmacytic lymphocytes. Over 80% of blood and bone marrow lymphocytes were CD5 +, CD19 +, CD20 +, CD23 + and CD38 +. Based on these findings, the diagnosis of B-CLL was made. The strong pruritus was resistant to antihistamines and steroids. Therefore, a trial with interferon (IFN) gamma 50 micrograms s.c. daily was started in order to suppress elevated serum IgE but failed. After 1.5 million units s.c. of IFN-alpha 2b every second day for 2 weeks, pruritus and serum IgE levels diminished markedly (48,000 IU/ml). Skin lesions, pruritus as well as skin infection and serum IgE level improved under continuing IFN-alpha 2b therapy for 2 years.


CONCLUSIONS
In cases with suspected hyper-IgE syndrome, hematological neoplasias have to be excluded. The results argue for a benefical effect of IFN-alpha 2b in suppressing IgE production and symptoms of pruritus in an early-stage B-CLL.
Neuber
K
K
Department of Dermatology, University Hospital Eppendorf, University of Hamburg, Germany.
Berg-Drewniock
B
B
Volkenandt
M
M
Neumaier
M
M
Zeller
W
W
Gross
G
G
Ring
J
J
D005434:Flow Cytometry; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood*; D007150:Immunohistochemistry; D016130:Immunophenotyping; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D007371:Interferon-gamma / Q000627:therapeutic use*; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011537:Pruritus / Q000150:complications* / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D011994:Recombinant Proteins
D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins; D007073:Immunoglobulin E; D007371:Interferon-gamma