Pituitary 2000;3:113–116 © Kluwer Academic Publishers. Boston. Printed in U.S.A.
Pituitary Apoplexy in a Patient with Acute Myeloid Leukemia and Thrombocytopenia Pituitary Apoplexy
Wongpraparut
N. Wongpraparut1, N. Pleanboonlers1, P. Suwattee1, P. Rerkpattanapipat1, A. Turtz2, M. Moster3, I. Gala4, and YN. Kim1 1Department
of Medicine, Albert Einstein Medical Center, Philadelphia, PA; 2Chief of Neurosurgery, Medical College of Pennsylvania Philadelphia, PA; 3Chairman, Department of Neurology, 4Director, Anatomic Pathology, Department of Pathology & Laboratory Medicine, Albert Einstein Medical Center, Philadelphia, PA.
Abstract. We describe a 72-year-old woman with a history of acute myeloid leukemia who developed pituitary apoplexy associated with thrombocytopenia secondary to chemotherapy. She presented with new onset severe headache, nausea, vomiting and blurred vision. Initial physical examination was unremarkable. CT scan of the head was initially negative. Upon admission for further work up, She developed a high-grade fever, hypotension and obtundation. Subsequent physical examination revealed bitemporal visual ~elds defects and decreased visual acuity. Repeat imaging of head revealed a hemorrhagic pituitary mass compressing the optic chiasm. Laboratory results were compatible with the diagnosis of pan-hypopituitary syndrome. She received high dose steroids and was transferred for transnasal sphenoidotomy decompression surgery. The visual defects improved postoperatively. A literature review of Pituitary apoplexy is presented. Pituitary apoplexy secondary to thrombocytopenia has never been reported. Keywords. pituitary apoplexy, thrombocytopenia, panhypopituitary syndrome, pituitary adenoma, acute myeloid leukemia
Introduction Pituitary apoplexy is a clinical syndrome of headaches, visual ~eld defects, ophthalmoplegia and alteration of consciousness resulting from hemorrhage or infarction of pituitary adenoma. Bailey ~rst described a case of a fatal hemorrhage in pituitary adenoma pathologically in 1898 [1], but the clinical syndrome was not recognized until 1950 when Brougham provided a review of reported cases to date and named the syndrome [2]. Since the original description, many authors have described pituitary apoplexy in association with a wide variety of medications, procedures, and pathologic states. We describe the ~rst case of pituitary apoplexy associated with thrombocytopenia.
Case presentation A 72-year-old woman with a history of acute myeloid leukemia, subtype M5B, had been receiving outpa-
tient consolidation chemotherapy with high dose cytarabine (Ara-C). She was found on surveillance testing to have a platelet count of 13,000. She was referred for admission for platelet transfusion. Six hours prior to admission, she developed severe headaches with nausea and vomiting. She described pain as a bilateral frontal headache with blurred vision in the left lateral ~eld. Initial physical examination as well as thorough neurological examination was unremarkable. She was sent for computed tomographic scan of the head, which was interpreted as negative. Upon admission for further work up, she developed a high fever, hypotension and obtundation over the next 12 hours. Subsequent physical examination showed severe bitemporal visual ~elds defects and decreased visual acuity (20/70 OD and 20/100 OS). Lumbar puncture was performed and revealed xanthochromic _uid. A magnetic resonance imaging of the head revealed an enlarging hemorrhagic pituitary mass with compression of the optic chiasm (Figs. 1A and 1B). Upon review of a previously done computed tomographic scan, a 1⫻1⫻1 cm adenoma of pituitary without bleeding (Fig. 2) was also noted. Laboratory results showed low serum level of GH (0.1 ng/ml), TSH (0.03 microU/ml) and prolactin (0.2 ng/ml), compatible with the diagnosis of pan-hypopituitary syndrome. (The cortisol level was not obtained due to prior steroid administration as part of the chemotherapy regimen.) High dose steroids were given to prevent adrenal crisis. The patient subsequently underwent surgical decompression of the mass via a transnasal sphenoidotomy. Postoperatively, she experienced prompt resolution of her headaches and marked improvement of the visual defect (visual acuity 20/60 OU). Tissue biopsy revealed, a pituitary adenoma, monomorphic sheet of
Address correspondence to: Nattawut Wongpraparut, MD, Department of Medicine, Albert Einstein Medical Center, 5401 Old York Road, Klein building, Philadelphia, PA 19141. E-mail:
[email protected] 113
Fig. 1A and 1B. Magnetic resonance imaging of the head revealed an enlarged hemorrhagic pituitary mass compressing the optic chiasma. 114
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Fig. 2. A computed tomographic scan of the head revealed a 1⫻1⫻1 cm adenoma of the pituitary gland without bleeding.
cells (unlike that of the normal gland), with moderate nuclear pleomorphism (Fig. 3). The cells had pale pink cytoplasms and moderate variation in nuclear size but no mitotic activity. Most of the tissue showed hemorrhagic infarction (Fig. 4) precluding further immuno-
histochemical characterization. No leukemic in~ltrate was demonstrated.
Discussion Intracranial bleeding should be suspected in any thrombocytopenic patients who develop new neuro-
Fig. 3. Hematoxylin and Eosin (H&E) stained section showing monomorphic tumor cells (upper left) with neuroendocrine features, undergoing early degeneration. (H&E, ⫻400)
Fig. 4. Coagulation necrosis of the adenoma showing sharp necrotic borders with cell ghosts. (H&E, ⫻400)
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logical ~ndings. We present the ~rst case report of pituitary apoplexy secondary to thrombocytopenia. The thrombocytopenia in our patient was caused by the use of the chemotherapeutic agent, cytarabine (1D-arabinofuranosyl cytosine, Ara-C), which has been known to cause severe myelosuppression, especially thrombocytopenia and leukopenia [3]. A pituitary adenoma is a highly vascularized tumor. Ischemic infarction of an adenoma may occur in an area that has outgrown its blood supply. Whereas early report suggested that pituitary apoplexy occurred primary in large macroadenoma with suprasellar extension [2], it was found that tumors of any size could undergo hemorrhage and apoplexy [4,5]. The incidence of hemorrhage into pituitary adenomas varied between 1.5% and 27.7% of cases in large surgical series [4–6]. There is no systematic analysis available regarding the prevalence of apoplexy in relation to tumor size. In a literature review of pituitary apoplexy related to anticoagulation, there was a case report by Nourzaden et al of a spontaneous hemorrhage into pituitary adenoma in the patient with an inferior wall myocardial infarction who was given coumadin [7]. The use of heparin in acute anticoagulation has been reported to trigger bleeding into pituitary adenoma [8]. Pituitary apoplexy following thrombolytic therapy has also been described [9, 10]. Pituitary apoplexy is most commonly discovered inpatients with known pre-existing pituitary adenoma; however, it may also be the ~rst clinical manifestation of an unrecognized pituitary tumor. A CT of the head is helpful in establishing the diagnosis of pituitary macroadenomas [11]. In addition, a MRI has also been shown to provide a correlation with operative ~ndings as described by Kurihara et al. [12]. Immediate administration of corticosteroids is mandatory due to the high incidence (66%) of acute adrenal insuf~ciency [13]. Neurosurgical decompression via a transphenoidal approach is the de~nitive therapy for pituitary decompression.
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