Case Presentation 5

Title, Authors and Institutions: / Hypereosinophilia with cutaneous involvement secondary to B-cell lymphoproliferation: A case report.
Byrne N 1, O’Dwyer M 2, Murphy A1. Correspondance:Dr. Niamh Byrne,
1 Department of Dermatology, University College Hospital Galway, Ireland.
2 Department of Haematology, University College Hospital Galway, Ireland.
Abstract:
(Your abstract must use Normal style and must fit in this space) / A 58-yr-old male presented with a 2 week history of a pruritic eczematous dermatitis, on a background of night sweats, fatigue and weight loss over the preceding 6 months. The rash started after completing a recent course of co-amoxiclav. The patient had no significant past medical history. Examination revealed an erythematous maculopapular rash on his face, trunk and limbs, in addition to inguinal, axillary and cervical lymphadenopathy. Laboratory investigations revealed leukophilia with significant eosinophilia [3.6 x 109/L (0.02 – 0.5)]. Differential diagnosis included a reactive drug rash, DRESS, viral infection, hypereosinophilic syndrome and malignancy.
Autoimmune and infective screen were negative. CT thorax, abdomen and pelvis disclosed splenomegaly and widespread mild lymphadenopathy. Skin biopsy demonstrated an eosinophil rich inflammation. Lymph node biopsy showed reactivity but no evidence of malignancy. Further investigations were performed to identify a potential haematological aetiology. Bone marrow aspirate showed an increased number of eosinophils. T-cell gene rearrangement studies were negative. Peripheral blood flow cytometry revealed clonal B cell lymphoproliferation suggestive of marginal zone or Mantle cell lymphoma. A diagnosis of hypereosinophilia secondary to a clonal B-cell lymphoproliferative disorder was made.
High dose systemic steroids were commenced and the patient’s cutaneous and systemic symptoms resolved. However, attempts to wean steroid therapy resulted in symptom recurrence. In consultation with the haematology specialists, the decision was made to target the low grade lymphoproliferation driving the hypereosinophilia. He was treated with 4 cycles of rituximab. Thereafter steroid treatment was successfully weaned, the rash resolved and his eosinophil count normalised. Follow up PET scan showed resolution of the lymphadenopathy and splenomegaly.
Hypereosinophilia can be idiopathic or secondary to a number of aetiologies including infection, atopy, autoimmune disease and malignancy [1]. Although acute elevation in eosinophils is not harmful, prolonged tissue infiltration causes damage. Therefore early diagnosis and treatment is vital in order to limit end-organ damage and prevent mortality. The skin is the most commonly affected organ but the heart, lungs and nervous system can also be involved. Hypereosinophilia has been associated with cutaneous T-cell lymphoma and abnormal T-cell clones in the blood via the release of eosinophilopoietic cytokines including IL-4 and IL-5 [2]. This case demonstrates that clonal B-cell proliferation may also cause hypereosinophilia and in such cases rituximab maybe a treatment option.
References:
[1]Tefferi A. Blood eosinophilia: a new paradigm in disease classification, diagnosis, and treatment. Mayo Clin Proc 2005; 80: 75 – 83.
[2] Simon HU, Plotz SG, Dummer R, et al. Abnormal Clones of T cells producing interleukin-5 in idiopathic eosinophilia. N Eng J Med 1999; 341:1112 – 1120.

Please return to closing date 26th August 2015