52-year-old man • hematemesis • history of cirrhosis • persistent fevers • Dx?

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doi:doi: 10.12788/jfp.0385

Case Reports

52-year-old man • hematemesis • history of cirrhosis • persistent fevers • Dx?

J Fam Pract. 2022 April;71(3):E17-E19 | doi: 10.12788/jfp.0385

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References

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5. Jacobs JL, Libby DM, Winters RA, et al. A cluster of Pneumocystis carinii pneumonia in adults without predisposing illnesses. N Engl J Med. 1991;324:246-250. doi: 10.1056/NEJM199101243240407

6. Ng VL, Yajko DM, McPhaul LW, et al. Evaluation of an indirect fluorescent-antibody stain for detection of Pneumocystis carinii in respiratory specimens. J Clin Microbiol. 1990;28:975-979. doi: 10.1128/jcm.28.5.975-979.1990

7. Cregan P, Yamamoto A, Lum A, et al. Comparison of four methods for rapid detection of Pneumocystis carinii in respiratory specimens. J Clin Microbiol. 1990;28:2432-2436. doi: 10.1128/jcm.28.11.2432-2436.1990

8. Turner D, Schwarz Y, Yust I. Induced sputum for diagnosing Pneumocystis carinii pneumonia in HIV patients: new data, new issues. Eur Respir J. 2003;21:204-208. doi: 10.1183/09031936.03.00035303

9. Smith RL, el-Sadr WM, Lewis ML. Correlation of bronchoalveolar lavage cell populations with clinical severity of Pneumocystis carinii pneumonia. Chest. 1988;93:60-64. doi: 10.1378/chest.93.1.60

10. Fleury-Feith J, Van Nhieu JT, Picard C, et al. Bronchoalveolar lavage eosinophilia associated with Pneumocystis carinii pneumonitis in AIDS patients. Comparative study with non-AIDS patients. Chest. 1989;95:1198-1201. doi: 10.1378/chest.95.6.1198

11. Thomas CF Jr, Limper AH. Current insights into the biology and pathogenesis of Pneumocystis pneumonia. Nat Rev Microbiol. 2007;5:298-308. doi: 10.1038/nrmicro1621

12. Toh BH, Roberts-Thomson IC, Mathews JD, et al. Depression of cell-mediated immunity in old age and the immunopathic diseases, lupus erythematosus, chronic hepatitis and rheumatoid arthritis. Clin Exp Immunol. 1973;14:193-202.

13. Mansharamani NG, Balachandran D, Vernovsky I, et al. Peripheral blood CD4 + T-lymphocyte counts during Pneumocystis carinii pneumonia in immunocompromised patients without HIV infection. Chest. 2000;118:712-720. doi: 10.1378/chest.118.3.712

14. McGovern BH, Golan Y, Lopez M, et al. The impact of cirrhosis on CD4+ T cell counts in HIV-seronegative patients. Clin Infect Dis. 2007;44:431-437. doi: 10.1086/509580

15. Bienvenu AL, Traore K, Plekhanova I, et al. Pneumocystis pneumonia suspected cases in 604 non-HIV and HIV patients. Int J Infect Dis. 2016;46:11-17. doi: 10.1016/j.ijid.2016.03.018

16. Albillos A, Lario M, Álvarez-Mon M. Cirrhosis-associated immune dysfunction: distinctive features and clinical relevance. J Hepatol. 2014;61:1385-1396. doi: 10.1016/j.jhep.2014.08.010

17. Lario M, Muñoz L, Ubeda M, et al. Defective thymopoiesis and poor peripheral homeostatic replenishment of T-helper cells cause T-cell lymphopenia in cirrhosis. J Hepatol. 2013;59:723-730. doi: 10.1016/j.jhep.2013.05.042

DISCUSSION

PCP typically occurs in immunocompromised individuals and may be related to HIV infection, malignancy, or exposure to immunosuppressive therapies.^1,2 While rare cases of PCP have been described in adults without predisposing factors, many of these cases occurred at the beginning of the AIDS epidemic, prior to reliable HIV testing.^3-5

Uncharted territory. We were confident in our diagnosis because immunofluorescence testing has very few false-positives and a high specificity.^6-8 But there were informational gaps. The eosinophilia recorded on BAL is poorly described in HIV-negative patients with PCP but well-described in HIV-positive patients, with the level of eosinophilia associated with disease severity.^9,10 Eosinophils are thought to contribute to pulmonary inflammation, which may explain the severity of our patient’s course.¹⁰

A first of its kind case?

To our knowledge, this is the first report of PCP in a patient with cirrhosis from chronic hepatitis C virus infection and no other predisposing conditions or preceding immunosuppressive therapy. We suspect that his lymphopenia, which was noted during his critical illness, predisposed him to PCP.

Individuals with lymphopenia and low CD4+ T-cell counts have been shown to be at increased risk of pneumocystis pneumonia.

Lymphocytes (in particular CD4+ T cells) have been shown to play an important role, along with alveolar macrophages and neutrophils, in directing the host defense against P jirovecii infection.^1,3,11 Individuals with lymphopenia and low CD4+ T-cell counts have been shown to be at increased risk of PCP; risk increases markedly with CD4+ T cells below 200 cells/µL.^11-13

Typical risk factors for lymphopenia had not been observed in this patient. However, cirrhosis has been associated with low CD4+ T-cell counts and disruption of cell-mediated immunity, even in HIV-seronegative patients.^14,15 There are several postulated mechanisms for low CD4+ T-cell counts in cirrhosis, including splenic sequestration, impaired T-cell production (due to impaired thymopoiesis), increased T-cell consumption, and apoptosis (due to persistent immune system activation from bacterial translocation and an overall pro-inflammatory state).^16,17

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