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. CONCLUSION: 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.
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. CONCLUSION: 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.