Hypersomatotropism Induced Secondary Polycythaemia Leading To Spontaneous Pituitary Apoplexy Resulting In Cure Of Acromegaly And Remission Of Polycythaemia: 'the Virightuous Circle'.
Patra, S., Biswas, S. N., Datta, J., & Chakraborighty, P. P. (2017). Hypersomatotropism induced secondary polycythaemia leading to spontaneous pituitary apoplexy resulting in cure of acromegaly and remission of polycythaemia: 'The virightuous circle'. BMJ case reports, 2017, bcr2017222669. https://doi.org/10.1136/bcr-2017-222669
View Original Source →Abstract
A young man with subtle clinical features suggestive of hypersomatotropism presented with acute-onset severe headache. Relevant investigations confirmed polycythaemia and growth hormone (GH)-secreting pituitary macroadenoma with apoplexy. Secondary polycythaemia and myeloproliferative disorders were ruled out. At follow-up after 3 months, resolution of polycythaemia and acromegaly was observed, evident on normal haemoglobin levels, a normocellular marrow, and normal insulin-like growth factor-1 (IGF-1) with glucose-suppressed GH levels. Direct mitogenic properties of GH-IGF-1 axis on bone marrow progenitor cells may very rarely lead to erythroid hyperplasia and subsequent polycythaemia, reversible with successful therapy of acromegaly. In this case, polycythaemia secondary to hypersomatotropism likely resulted in pituitary apoplexy with subsequent remission of both acromegaly and resultant polycythaemia.
Case Details
Disease Location
Hypothalamo-pituitary macroadenoma abutting optic chiasm
Personal Characteristics
36-year-old
Clinical Characteristics
Presents with 5 days of sudden onset severe holocephalic headache. P/e: gcs 15, mild prognathism, prominent supraorbital ridges, broad nose, thick lips, plethora of bilateral lower palpebral conjunctiva, hyperhidrosis, oily skin, spade-shaped large hands and large feet. Labs showed polycythemia with normal wbcs and platelets.true erythrocytosis was confirmed by an increased red cell mass. Bone marrow biopsy examination revealed hypercellular marrow with erythroid hyperplasia. MRI of hypothalamo-pituitary region revealed a sellar mass (26 mm× 21 mm× 15 mm) abutting the optic chiasma, but without any enhancement on gadolinium contrast. The mass was hyperintense in t1-weighted and hypointense in t2-weighted sequences suggestive of pituitary macroadenoma with apoplexy. Endocrinological evaluation established hypersomatotropism with multiple pituitary hormones deficiencies.
Remission Characteristics
Underwent phlebotomy with with removal of 300 ml of venous blood for polycythemia. Compression stockings and intermittent pneumatic compression device were used for vte prophylaxis. Endocrine management with intravenous hydrocortisone (200 mg/day in four divided dosages) initially, followed by oral hydrocortisone (15 mg/day) and levothyroxine (50 mcg/day). Three months after his initial presentation, conjunctival plethora and hyperhidrosis subsided while a repeat bone marrow biopsy documented a normocellular marrow. In view of pituitary apoplexy and subsequent normal insulin-like growth factor-1 (igf-1) levels with glucose-suppressed growth hormones (gh) at 3 months, therapy for acromegaly (either surgical or medical) was not considered.
Treatment & Mechanisms
Proposed Remission Mechanisms
Explained by pituitary apoplexy which was likely a manifestation of polycythaemia that led to cure of acromegaly, which in turn resulted in remission of polycythaemia.
Clinical Treatment
Peripheral phlebotomy, endocrine management with intravenous hydrocortisone (200 mg/day in four divided dosages) initially, followed by oral hydrocortisone (15 mg/day) and levothyroxine (50 mcg/day).