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Aggressive cutaneous vasculitis in a patient with chronic lymphatic leukemia following granulocyte colony stimulating factor injection: a case report
© El Husseiny and Mattar; licensee BioMed Central Ltd. 2011
Received: 23 January 2009
Accepted: 1 March 2011
Published: 1 March 2011
Vasculitis has been reported in a few cases of chronic lymphatic leukemia and with granulocytic colony-stimulating factor therapy. Those with granulocytic colony-stimulating factor occurred after prolonged therapy and there was a rise in total leukocyte count unlike that in our patient who received just a single injection for the first time.
We report the case of a 64-year-old Egyptian man with chronic lymphatic leukemia who developed progressive cutaneous vasculitic lesions following injection of a single dose of a granulocytic colony stimulating factor before a third cycle of chemotherapy to improve neutropenia. This is an unusual case and the pathogenesis is not fully understood. Our patient was not on any medical treatment except for bisoprolol for ischemic heart disease. Although aggressive management with steroids, anticoagulation and plasmapheresis had been carried out, the condition was aggressive and the patient's consciousness deteriorated. A magnetic resonance imaging scan of his brain revealed multiple ischemic foci that could be attributed to vasculitis of the brain.
The aim of this case report is to highlight the importance of monitoring patients on granulocytic colony-stimulating factor therapy, especially in the context of other conditions (such as a hematological malignancy) that may lead to an adverse outcome.
An adverse reaction has been reported in rare cases with granulocyte colony-stimulating factor treatment and in patients with hematological malignancies. Here we present a case of chronic lymphatic leukemia in which the patient received just one injection of lenograstin for neutropenia before starting the third cycle of chemotherapy, in the absence of other medical conditions. After the injection he developed cutaneous lesions and a skin biopsy revealed vasculitis. The condition was severe and the patient died 15 days after the onset of symptoms.
This case is considered unusual and the pathogenesis is not fully understood as the described vasculitis does not typically develop in this context with granulocytic colony-stimulating factor (G-CSF) treatment. Cutaneous vasculitis was reported in less than one in 7000 of those treated with G-CSF, and was moderate to severe in intensity. Most patients had severe chronic neutropenia and were on long-term G-CSF therapy. Symptoms developed with an increase in the absolute neutrophilic count (ANC) and stopped when the ANC decreased and the patients continued treatment at a reduced dosage . Regarding the association of vasculitis with hematological malignancies, in a study done on 95 patients with hematological malignancy it was found that 22% had cutaneous vasculitis, 39% developed it concomitantly with malignancy, 26% of these before development of a malignancy and 35% after diagnosis of a malignancy. In 61% the vasculitis was explained only by the malignancy and in the remaining 39% it was explained by other factors, which included infection, cryoglobinemia and medications . The association between lymphoma and vasculitis is rare in general. Lymphoproliferative disorders are the most common hematological malignancies associated with vasculitis (20%) especially hairy cell leukemia. Cases of vasculitis with CLL are rare (0.1 to 2%) . The mechanisms of development of vasculitis in CLL include 1) immune complexes, 2) activation of B-lymphocytes, 3) antibodies directed toward endothelial antigens, 4) the direct effect of a malignancy on the vascular wall, 5) adverse reactions to anticancer drugs, and 6) CD5-positive B cells present in CLL may produce auto antibodies and monoclonal immunoglobulins with various autoantibody activities .
In conclusion, patients should be monitored for development of inflammatory processes during G-CSF therapy, which should be given with caution especially in hematological malignancy situations.
Written informed consent was obtained from the patient's next of kin for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We acknowledge Dr Galal El Enany, professor of dermatology at Cairo University, who analysed the skin biopsy.
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