The syndrome of multiple endocrine neoplasia type IIA in three generations of one family (continued 50­years of observation)

We present a clinical case of 22yo patient with diagnosis of pheochoromocytoma complicated with myelodysplastic syndrome as manifestation of paraneoplastic phenomenon. The onset of the disease displays typical clinical picture of pheochromacytoma. After medical examination and survey it was discovered a tumor in left adrenal gland and elevation of metanephrines and normetanephrines. In addition, patient has severe anemia and thrombocytopenia. Sternal punction with morphological examination and immunophenotyping were performed. Patient had consulted by hematologist. After all additional analysis cause of anemia and thrombocytopenia remained unknown and related to presence of tumor.Patient was performed a long-time and vast pre-operative preparation with administration of doxazosin and transfusions of blood and platelet concentrate. In continuation, mass in left adrenal gland was excised with spleen by thoracophrenolaparotomy. Morphology confirmed pheochromacytoma, which has typical histological structure and circulatory disorders. Spleen has no specifical features except of focuses of extramedullar hematopoiesis. Patient has short period of hormone therapy to avoid adrenal crisis on post-operative stage. Also massive transfutions of blood and platelet concentrate was performed due to persisting anemia and thrombocytopenia. There were no manifestations of hemorrhage syndrome after the surgery. Myelodysplastic syndrome was detected 3 month later and manifested itself in form of refractory anemia and severe thrombocytopenia, which persisted for long period and required corrections with therapy and transfusions. Only through 5-year observation after adrenalectomy patient has positive dynamic and leveling of laboratory tests without symptoms of myelodysplastic syndrome.

show abstract

Pheochromocytoma with paraneoplastic phenomena manifested as myelodysplastic syndrome

Sergiyko

Korotovsky

Tul`ganova

et al. 2023

Endo Serg

View full text Add to dashboard Cite

show abstract

scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.

Contact Info

customersupport@researchsolutions.com

10624 S. Eastern Ave., Ste. A-614

Henderson, NV 89052, USA

This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.

Blog Terms and Conditions API Terms Privacy Policy Contact Cookie Preferences Do Not Sell or Share My Personal Information

Made with 💙 for researchers

Part of the Research Solutions Family.

The syndrome of multiple endocrine neoplasia type IIA in three generations of one family (continued 50years of observation)

Cited by 1 publication

References 5 publications

Pheochromocytoma with paraneoplastic phenomena manifested as myelodysplastic syndrome

Pheochromocytoma with paraneoplastic phenomena manifested as myelodysplastic syndrome

Contact Info

Product

Resources

About

The syndrome of multiple endocrine neoplasia type IIA in three generations of one family (continued 50­years of observation)

Cited by 1 publication

References 5 publications

Pheochromocytoma with paraneoplastic phenomena manifested as myelodysplastic syndrome

Pheochromocytoma with paraneoplastic phenomena manifested as myelodysplastic syndrome

Contact Info

Product

Resources

About

The syndrome of multiple endocrine neoplasia type IIA in three generations of one family (continued 50years of observation)