Sheehan's syndrome with pancytopenia: a case report and review of the literature
© Fatma et al; licensee BioMed Central Ltd. 2011
Received: 10 November 2010
Accepted: 3 October 2011
Published: 3 October 2011
Sheehan's syndrome is defined by varying degrees of anterior pituitary deficiency due to postpartum ischemic necrosis of the pituitary gland after massive bleeding. It is a rare disorder in western countries and even in Tunisia. Hematologic abnormalities such as normochromic anemia have been reported in these patients. However, pancytopenia is rarely observed.
We describe the case of a 48-year-old Tunisian woman with features of hypopituitarism. Laboratory tests showed pancytopenia that was completely reversed after adequate hormone replacement.
Clinicians should consider the possibility of hypopituitarism as a cause of pancytopenia. This is an original case report that is of interest to hematologists, who should be aware of Sheehan's syndrome as a treatable etiology of pancytopenia for women.
Sheehan's syndrome is characterized by varying degrees of anterior pituitary dysfunction due to postpartum ischemic necrosis of the pituitary gland after massive bleeding. The most frequent hematologic finding is anemia. Pancytopenia is rarely observed in patients with Sheehan's syndrome. Only seven cases have been reported up to now.
Hematological anomalies in previously published cases and our patient 
Our patient was followed for six weeks after which a complete hematological recovery was noted, with a eucortisolemic and euthyroid state. In fact, the hematologic abnormality had dramatically improved. Her leukocyte count was 6.2 × 109/L, her hemoglobin level was 10 g/dL and her platelet count was 219 × 109/L, all within the normal range.
The diagnosis of Sheehan's syndrome is determined by the patient's history and physical examination, and confirmed by laboratory tests (hormone levels and hormone stimulation tests which prove anterior pituitary failure). Laboratory tests can reveal many other anomalies such as hyponatremia. This is the most common electrolyte imbalance, occurring in 33% to 69% of cases [1, 2]. Cortisol deficiency, hypothyroidism and volume depletion are the main causes of hyponatremia. It also seems that Sheehan's syndrome has hematological consequences, to which little attention is paid because of their rarity. Anemia is well recognized as a feature of hypopituitarism. Gokalp et al. have recently reported hematological abnormalities in 65 patients with Sheehan's syndrome, 80% of whom presented with anemia, compared with 25% of controls . Many hormonal deficiencies, such as hypothyroidism, adrenal insufficiency and gonadal hormonal deficiency, can explain normochromic anemia in hypopituitarism . On the other hand, it can be the result of a physiologic adjustment to lower oxygen requirement, as pituitary hormones modulate the production of erythropoietin in the kidney . The low erythropoietin levels found in these patients support this argument. However, within the framework of hematologic disorders, pancytopenia is rarely observed in patients affected with Sheehan's syndrome.
Hormonal results of all reported patients 
Pancytopenia is a rare appearance of a hormonal abnormality; clinicians should consider the possibility of hypopituitarism as a cause of pancytopenia and indicate a series of hormonal examinations. Multiple anterior pituitary hormone deficiencies in Sheehan's syndrome can be responsible for pancytopenia. A simple replacement therapy with thyroid and cortisol hormones results in complete recovery. So, hematologists need to be aware of Sheehan's syndrome as a treatable etiology of pancytopenia in women.
Since the postpartum I suffered from fatigue, pallor, anemia and amenorrhea. Recently I presented with vomiting and diarrhea. Biological findings showed pancytopenia and hypopituitarism. After starting hormone replacement treatment, I felt better. I prefer to remain anonymous.
Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Anfuso S, Patrelli TS, Soncini E, Chiodera P, Fadda GM, Nardelli GB: A case report of Sheehan's syndrome with acute onset, hyponatremia and severe anemia. Acta Biomed. 2009, 80 (1): 73-76.PubMedGoogle Scholar
- Huang YY, Ting MK, Hsu BR, Tsai JS: Demonstration of reserved anterior pituitary function among patients with amenorrhea after postpartum hemorrhage. Gynecol Endocrinol. 2000, 14 (2): 99-104. 10.3109/09513590009167667.View ArticlePubMedGoogle Scholar
- Gokalp D, Tuzcu A, Bahceci M, Arikan S, Bahceci S, Pasa S: Sheehan's syndrome as a rare cause of anemia secondary to hypopituitarism. Ann Hematol. 2009, 88 (5): 405-410. 10.1007/s00277-008-0607-4.View ArticlePubMedGoogle Scholar
- Kim DY, Kim JH, Park YJ, Jung KH, Chung HS, Shin S, Yun SS, Park S, Kim BK: Case of complete recovery of pancytopenia after treatment of hypopituitarism. Ann Hematol. 2004, 83 (5): 309-312. 10.1007/s00277-003-0800-4.View ArticlePubMedGoogle Scholar
- Laway BA, Bhat JR, Mir SA, Khan RS, Lone MI, Zargar AH: Sheehan's syndrome with pancytopenia--complete recovery after hormone replacement (case series with review). Ann Hematol. 2010, 89 (3): 305-308. 10.1007/s00277-009-0804-9.View ArticlePubMedGoogle Scholar
- Ferrari E, Ascari E, Bossolo PA, Barosi G: Sheehan's syndrome with complete bone marrow aplasia: long-term results of substitution therapy with hormones. Br J Haematol. 1976, 33 (4): 575-582. 10.1111/j.1365-2141.1976.tb03576.x.View ArticlePubMedGoogle Scholar
- Ozdogan M, Yazicioglu G, Karadogan I, Cevikol C, Karayalcin U, Undar L: Sheehan's syndrome associated with pancytopenia due to marrow aplasia: full recovery with hormone replacement therapy. Int J Clin Pract. 2004, 58 (5): 533-535. 10.1111/j.1368-5031.2004.00059.x.View ArticlePubMedGoogle Scholar
- Akoz AG, Atmaca H, Ustundag Y, Ozdamar SO: An unusual case of pancytopenia associated with Sheehan's syndrome. Ann Hematol. 2007, 86 (4): 307-308. 10.1007/s00277-006-0233-y.View ArticleGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.