A 36-year-old female with hemoglobin Nottingham (betaFG 5(98) Val → Gly) causing severe hemolytic anemia and chronic thromboembolic pulmonary hypertension presented with symptomatic subacute right lower lobar pulmonary arterial thrombosis requiring surgical pulmonary thrombectomy. We describe a successful, multidisciplinary approach to the problems associated with this disease, particularly with the use of cardiopulmonary bypass and deep hypothermic circulatory arrest. Perfusion (2007) 22, 299—301.
Get full access to this article
View all access options for this article.
References
1.
Rother RP, Bell L., Hillmen P., Gladwin MTThe clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. JAMA2005; 293: 1653—62.
2.
Orringer EP, Felice A., Reese A. et al. Hemoglobin Nottingham (alpha2beta2 (FG5) 98 val leads to gly) in a Caucasian male: clinical and biosynthetic studies. Hemoglobin1978; 2: 315—32.
3.
Gordon-Smith EC, Dacie JV, Blecher TE, French EA, Wiltshirre BG, Lehmann H.Haemoglobin Nottingham, beta FG 5 (98) valgly: a new unstable hemoglobin producing severe haemolysis. Proc R Soc Med1973; 66: 507—8.
4.
Brabec V., Indrak K., Fortova H. et al. Hemoglobin Nottingham or alpha 2 beta 2 98 (FG5) Val→Gly in a Czech child. Ann Hematol1994; 69: 93—5.
5.
Cepreganova B., Wilson JB, Huisman TH, Hume HAHemoglobin Nottingham or alpha 2 beta 2(98) (FG5) Val— Gly observed as a de novo mutation in a Canadian child. Hemoglobin1992; 16: 77—9.
6.
Fedullo PF, Auger WR, Kerr KM, Rubin LJChronic thromboembolic pulmonary hypertension. N Engl J Med2001; 345: 1465—72.
7.
Murali B., Drain A., Seller D., Dunning J., Vuylsteke A.Pulmonary thromboendarterectomy in a case of hereditary stomatocytosis. Br J Anaesth2003; 91: 739—41.
8.
Feinsten JA, Goldhaber SZ, Lock JE et al. Balloon pulmonary angioplasty for treatment of chronic thromboembolic pulmonary hypertension. Circulation2001; 103: 10—13.
9.
Englert SJ, Jiang J.Aortic valve replacement for a patient with essential thrombocythemia: a case report. J Extra Corpor Technol2004; 36: 166—8.
10.
Hankins JS, Ware RE, Rogers ZR et al. Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study. Blood2005 Oct 1; 106(7): 2269—75.
11.
Rose C., Bauters F.Hydroxyurea therapy in highly unstable hemoglobin carriers. Blood1996; 88: 2807—8.
