- Department of Neurosurgery, Faculty of Clinical Medicine, University of the Ryukyus, Nakagami-gun, Okinawa, Japan
- Department of Neurosurgery and Pathology, Kiryu Kousei Hospital, Kiryu, Gunma, Japan
Department of Neurosurgery, Faculty of Clinical Medicine, University of the Ryukyus, Nakagami-gun, Okinawa, Japan
DOI:10.4103/2152-7806.73318© 2010 Watanabe T This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Watanabe T, Kurihara H, Magarisawa S, Shimoda S, Yoshida K, Ishiuchi S. Resolution of immune thrombocytopenic purpura associated with extranodal B-cell lymphoma of the petroclival region after radiotherapy. Surg Neurol Int 27-Nov-2010;1:76
How to cite this URL: Watanabe T, Kurihara H, Magarisawa S, Shimoda S, Yoshida K, Ishiuchi S. Resolution of immune thrombocytopenic purpura associated with extranodal B-cell lymphoma of the petroclival region after radiotherapy. Surg Neurol Int 27-Nov-2010;1:76. Available from: http://sni.wpengine.com/surgicalint_articles/resolution-of-immune-thrombocytopenic-purpura-associated-with-extranodal-b-cell-lymphoma-of-the-petroclival-region-after-radiotherapy/
Background:Secondary immune thrombocytopenic purpura (ITP) associated with extranodal B-cell non-Hodgkin’s lymphoma (NHL) is extremely rare. The optimal management is not established. We report a first case of ITP in association with extranodal B-cell NHL originating in the lower petroclival region, successfully managed by local tumor control using conventional radiotherapy.
Case Description:A 75-year-old man presented with a two-month history of hearing loss, hoarseness, and dysphagia. Neuroimaging revealed a large enhanced lesion in the left lower petroclival bone near the jugular foramen. Isolated unilateral parotid lymphadenopathy was also noted. Preoperative laboratory findings were normal, except for elevation of serum soluble interleukin-2 receptor level. A suboccipital craniotomy was performed and a biopsy sample was taken. Histological and immunohistochemical examination confirmed small B-cell lymphoma with plasmacytic differentiation. After initiation of radiotherapy, thrombocytopenia (24,000/µl) rapidly developed. Serological and bone marrow examination confirmed ITP. Prednisone was given at 1 mg/kg/day and radiation therapy was continued. After more than 32Gy, platelet count rapidly normalized. Radiotherapy to the tumor site achieved local tumor control and ITP was resolved. No evidence of recurrence and normal platelet count were confirmed at the two-year follow-up examination.
Conclusion:Local control of the tumor was considered important in the resolution of secondary ITP in association with extranodal NHL of the skull base region.
Keywords: B-cell lymphoma, immune thrombocytopenic purpura, radiotherapy, skull base
Immune thrombocytopenic purpura (ITP) is a type of autoimmune thrombocytopenia associated with antibody-mediated accelerated platelet destruction, characterized by low platelet count and hemorrhagic tendency in the skin or submucosa despite normal or overactive platelet production.[
We describe a first case of secondary ITP associated with lower petroclival B-cell NHL, which was successfully managed by local tumor control using conventional radiotherapy.
A 75-year-old male presented with loss of hearing, hoarseness, and dysphagia, progressively deteriorating over two months. The patient had a past history of diabetes mellitus and hypertension. Clinical examination found a painless left parotid lymphadenopathy and a paralysis of the soft palate and tongue on the affected side. Audiography showed Class 2 hearing on the Gardner–Robertson scale[
A suboccipital craniotomy was performed and a biopsy sample was taken. The tumor had infiltrated into the suboccipital muscles and eroded through the occipital bone and petrous bone. The dura mater was not involved. Histological analysis of the biopsy samples demonstrated the diffusely infiltrated tumor in the muscles and bone, which consisted of small to medium lymphocytes and plasmacytoid cells with abundant basophilic cytoplasm and lymphocyte-like nuclei [
The postoperative course was uneventful. Laboratory examination just after surgery found no abnormalities. Local radiation therapy at 2Gy/fraction was started one week after surgery. Parotid lesion was also included in the field of radiation. Complete blood count after 14Gy irradiation revealed thrombocytopenia with platelet count of 38,000/µl, hemoglobin of 11.7 g/dl, and white blood cell count of 5200/µl. On the next day, platelet count fell to 24,000/µl. Serum biochemistry and serum protein electrophoresis revealed findings within normaL limits. Serum M-component was not detected. Bence–Jones protein was not found in the urine. Platelet-associated immunoglobulin G was 254 ng/107cells (normal range 9–25 ng/107cells). Other autoantibodies including anticardiolipin and lupus anticoagulant were not detected. Serological tests for recent viral infection (hepatitis B virus, hepatitis C virus) were negative. Results of bone marrow aspiration showed normal cellularity and increase in megakaryocytes. Drug-induced thrombocytopenia was also excluded. The diagnosis was immune thrombocytic purpura. Oral prednisone 1 mg/kg/day was initiated and was gradually tapered within a few weeks. Platelet count rose to 90,000/µl and 220,000/µl after 32Gy and 46Gy irradiation, respectively. Finally, a total dose of 50Gy was delivered to the entire lesion, and a booster dose of 6Gy was added to the residual lesion in the clivus. Hoarseness and dysphagia improved to allow eating of normal food. Magnetic resonance imaging after radiotherapy confirmed complete remission [
Acquired ITP has developed in association with various NHL subtypes, including small lymphocytic lymphoma, follicular lymphoma, marginal cell lymphoma, mantle cell lymphoma, hairy cell lymphoma, lymphoplasmacytic lymphoma, and high grade B-cell lymphoma.[
Malignant lymphoma may involve extranodal sites of the head and neck region, and arise most commonly in association with Waldeyer’s ring and ocular adnexal structures.[
The present case of ITP associated with extranodal B-cell lymphoma originating in the lower petroclival lesion was resolved after local tumor control achieved by conventional radiotherapy. Local tumor control may be important in treating ITP associated with localized indolent extranodal NHL.
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