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

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


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


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.

Diabetology Endocrinology Internal medicine

Article was published in

Internal Medicine

Issue 6

2010 Issue 6

Most read in this issue

This topic is also in:

Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account