Ann Thorac Surg 2009;88:e37-e38. doi:10.1016/j.athoracsur.2009.07.031
© 2009 The Society of Thoracic Surgeons
Case Reports
Lung Resection for Treatment of Idiopathic Thrombocytopenic Purpura Associated With a Pulmonary Lymphoma
Hany Elsayed, FRCS(Cth)a,*,
Mohamed Hassan, MRCSb,
John Nash, FRCPathc,
Matthew Lyall, MBBSc,
Michael Poullis, FRCS(Cth)a
a Cardiothoracic Department, Liverpool Heart and Chest Hospital, Liverpool, United Kingdom
b Cardiothoracic Department, Blackpool Victoria Hospital, Blackpool, United Kingdom
c Pathology Department, Royal Liverpool University Hospital, Liverpool, United Kingdom
Accepted for publication July 1, 2009.
* Address correspondence to Dr Elsayed, Liverpool Heart and Chest Hospital, Thomas Dr, Liverpool, L14 3PE, United Kingdom (Email: hanyhassan77{at}hotmail.com).
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Abstract
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Most primary lymphomas of the lung arise from the mucosa-associated lymphoid tissue of the bronchus. Autoimmune phenomena are associated with non-Hodgkin's lymphoma; among them immune thrombocytopenia is one of the most rare. In this study we report an extremely rare case of a patient with primary pulmonary mucosa-associated lymphoid tissue lymphoma associated with autoimmune thrombocytopenic purpura, with rapid restoration of normal platelet count after lung resection.
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Introduction
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Extra nodal lymphomas are most frequently found in the gastrointestinal tract, but primary pulmonary lymphomas are extremely rare. Most primary pulmonary lymphomas arise from the mucosa-associated lymphoid tissue (MALT) [1].
Autoimmune phenomena are associated with non-Hodgkin's lymphoma. Among these phenomena, hematological autoimmune diseases are a special subgroup and include autoimmune hemolytic anemia autoimmune thrombocytopenia (ITP) and Evans's syndrome [2].
We present the first reported case of a patient with primary pulmonary MALT lymphoma associated with autoimmune thrombocytopenic purpura with rapid restoration of normal platelet count after lung resection.
A 66-year-old woman was referred for consideration of lung resection. She had been complaining of persistent myalgia and fatigue for the past 3 years, which were particularly worse in the past 2 months. She has mild dyspnea with a New York Heart Association functional classification of II, due to mild chronic obstructive pulmonary disease for which she is using medical inhalers. Her social history included being an ex-smoker with a 30-pack/year history of cigarette smoking.
Her past medical history included tuberculosis in childhood, hiatus hernia, hysterectomy for fibroids, and a hip replacement 3 years ago. A vasculitic screen was negative, but a complete blood count showed marked thrombocytopenia with a platelet count of 53 x 109/L.
As part of her investigations she had a chest roentgenogram that showed a suspicious mass in her left lung. Consequently, she had a computed tomographic scan (Fig 1) that showed a 36-mm soft tissue density in the left upper lobe. Another area of soft tissue density of unidentified cause was found adjacent to the horizontal fissure on the right side and was noted.
A positron emission tomographic fluorodeoxyglucose scan shows avid uptake in the left upper lobe (standard uptake value, 8) with no evidence of uptake elsewhere, which is consistent with stage I malignancy. Her spirometry showed a forced expiratory volume of 1 second of 1.68 (70% of predicted) with a transfer factor of 54% of predicted.
In view of a possible thoracotomy, and given her low platelet count due to ITP, prednisolone (50 mg/day) was administered, but unfortunately her platelet count dropped to 23 x 109/L and then 9 x 109/L within 2 weeks. Platelet increments were given with no response. Finally immunoglobulins were administered and succeeded to boost her platelet count to 120 x 109/L, which was believed to be acceptable to perform surgery.
She had a left muscle-sparing thoracotomy and an uneventful left upper lobectomy using single-lung ventilation. Three days after surgery her platelet count was climbing to 230 x 109/L. Her postoperative course was uneventful. She was discharged home 4 days after her operation.
Her histopathology report was macroscopically a 45-mm mass reaching the pleural surface but not invading it, and microscopically a nodular-follicular lymphoid architecture composed mainly of small lymphocytes with occasional plasma cells (Fig 2). Lympho-epithelial lesions were present, and follicular colonization by MALT lymphoma cells was confirmed on immunohistochemical staining (Fig 3). Overall, the appearances were compatible with a pulmonary B-cell non-Hodgkin lymphoma, which was specifically an extranodal marginal zone lymphoma (MALToma).
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Fig 2. Histopathology showing lymphomatous infiltration of the lung with detail of the neoplastic lymphocytes (Hematoxylin and eosin; x200).
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Fig 3. Immunohistochemical study using Ki67 proliferation marker showing high turnover in the residual follicle centers, but a low rate in the lymphoma cells (x100).
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A review in the clinic at 2 and 6 weeks after surgery revealed her platelet count to be 623 x 109/L and 650 x 109/L, respectively. Our oncologists suggested that there was no need for adjuvant chemotherapy. Sixteen months later, she was totally asymptomatic with a normal platelet count.
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Comment
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Immune thrombocytopenia of malignancy is most frequently associated with lymphoproliferative disorders. Thrombocytopenia generally does not develop in the MALT subtype of lymphoma (MALToma) unless the tumor has infiltrated the bone marrow. Cytopenias resulting from immunologic causes in patients with MALT lymphoma were rarely reported in the literature [3].
The ITP is associated with production of anti-platelet antibodies. Sakai and colleagues [4] have reported that immunoglobulin G-k type M-protein is produced by MALT lymphoma and is the culprit in causing ITP. However, the details of the mechanism remain unclear [4].
Primary pulmonary MALT lymphoma is diagnosed with pathologic examination and immunohistochemical studies usually after exploratory thoracotomy, as in our case. Kamiuehera and colleagues [5] summarized 53 cases of primary pulmonary lymphoma in Japan and found that definitive diagnosis was established during and after surgery in 72.2% of patients, with 5.6% at autopsy and 20.3% with transbronchial and percutaneous biopsy [5].
The prognosis of primary pulmonary lymphoma is generally favorable in most series, with a 5-year survival rate of more than 80%, and a median survival time of more than 10 years [5]. Although adjuvant chemotherapy was given in all three reported cases of ITP associated with gastric MALToma to achieve complete remission of ITP, as with most cases, there was resistance to the steroid therapy [6] similar to our case. It was believed that this was not justified here, due to lack of any evidence and the fact that the patient had restored her normal platelet count shortly after lung resection.
In conclusion, this rare case illustrates the fact of the existing relation between primary pulmonary MALToma and ITP. Patients are best managed preoperatively with immunoglobulins to improve their platelet count. Surgical resection is the treatment of choice for these cases.
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References
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- Paquale F, Victor FT, Harkiran A, Claude D, Mark SA, Peter CP. Non-Hodgkin's lymphoma of the lung Ann Thorac Surg 2000;69:993-997.[Abstract/Free Full Text]
- Sakalli H, Akcali Z, Kayaselcuk F, Ozyilkan O. Maltoma presenting with thrombocytopenia Am J Clin Oncol 2005;28:321-322.[Medline]
- Jardin F, Levesque H, Tilly H. Autoimmune manifestations in Non-Hodgkin's lymphoma Rev Med Interne 2005;26:557-571.[Medline]
- Sakai A, Katayama Y, Mizuno A, et al. A MALT lymphoma producing IgG-k type M-protein Rinsho Ketueski 2000;41:658-663.
- Kamiuehera Y, Hirai T, Kawashima O, Morishita Y. Primary pulmonary malignant lymphoma of mucosa associated lymphoid tissue: a case report with review of Japanese literature Nippon Kyobu Geka Gakkai Zasshi 1997;45:1620-1626.[Medline]
- Hauswirth AW, Skrabs C, Schützinger C, et al. Autoimmune thrombocytopenia in non-Hodgkin's lymphomas Haematologica 2008;93:447-450.[Abstract/Free Full Text]
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Abstract
Introduction
