Pulmonary hypertension – unusual complication of haemolysis and the bacterial overgrowth syndrome

Czech version

Authors: V. Kojecký; M. Lukaštíková
Authors‘ workplace: Interní klinika IPVZ Praha, Krajská nemocnice T. Bati, a. s., Zlín, přednosta doc. MU Dr. Čestmír Čihalík, CSc.
Published in: Vnitř Lék 2010; 56(6): 513-516
Category: Case Reports

Overview

The authors describe a case of a patient with celiac disease in whom a secondary bacterial overgrowth had developed in the small intestine. Vitamin B₁₂ and folic acid consumption deficiency resulted in a development of macrocytary anaemia and non‑immune haemolysis. Subsequently, within a few weeks, a significant pulmonary hypertension has developed, the cause of which was first unclear. Haemolysis as well as pulmonary hypertension ceased following bacterial overgrowth treatment and B₁₂ and, later on, folic acid substitution. Retrospectively, the authors infer that this could have been the haemolytic anaemia‑associated pulmonary hypertension syndrome.

Key words:
pulmonary hypertension –⁠ haemolysis –⁠ celiac disease

Sources

1. Halfdanarson TR, Litzow MR, Murray JA. Hematologic manifestations of celiac disease. Blood 2007; 109 : 412 –⁠ 421.

2. Hoffbrand AV, Tabaqchali S, Booth CC et al. Small intestinal bacterial flora and folate status in gastrointestinal disease. Gut 1971; 12 : 27 –⁠ 33.

3. Klipstein FA, Samloff IM. Folate synthesis by intestinal bacteria. Am J Clin Nutr 1966; 19 : 237 –⁠ 246.

4. Jansa P, Aschermann M, Riedel M et al. Doporučení pro diagnostiku a léčbu plicní arteriální hypertenze v ČR. Cor Vasa 2004; 46: K35 –⁠ K44.

5. Castro O, Hoque M, Brown BD. Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood 2003; 101 : 1257 –⁠ 1261.

6. Řiháček I, Souček M, Kára T et al. Primární plicní hypertenze –⁠ aktuální pohled na diagnostiku a léčbu. Vnitř Lék 2003; 49 : 482 –⁠ 489.

7. Rother RB, Bell L, Hillmen P et al. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. JAMA 2005; 293 : 1653 –⁠ 1662.

8. Gladwin MT, Sachdev V, Jison ML et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004; 350 : 886 –⁠ 895.

9. Morris CR. Mechanisms of vasculopathy in sickle cell disease and thalassemia. Hematology Am Soc Hematol Educ Program 2008; 1 : 177 –⁠ 185.

10. Morris CR, Gladwin MT, Kato GJ. Nitric oxide and arginine dysregulation: a novel pathway to pulmonary hypertension in hemolytic disorders. Curr Mol Med 2008; 8 : 620 –⁠ 632.

11. Tofovic SP, Jackson EK, Rafikova O. Adenosine deaminase -⁠ adenosine pathway in hemolysis‑associated pulmonary hypertension. Med Hypotheses 2009; 72 : 713 –⁠ 719.

12. Slebos DJ, Ryter SW, Choi AM. Heme oxygenase -⁠ 1 and carbon monoxide in pulmonary medicine. Respir Res 2003; 4 : 7.

13. Kato GJ, Gladwin MT. Evolution of novel small‑molecule therapeutics targeting sickle cell vasculopathy. JAMA 2008; 300 : 2638 –⁠ 2646.