Idiopathic CD4 Lymphocytopenia (HIV-Negative AIDS): Case Report and Literature Review
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CASE REPORT AND LITERATURE REVIEW
P: 24-24
January 2019

Idiopathic CD4 Lymphocytopenia (HIV-Negative AIDS): Case Report and Literature Review

Mediterr J Infect Microb Antimicrob 2019;8(1):24-24
1. Karadeniz Technical University Faculty of Medicine, Department of Infectious Diseases and Clinical Microbiology, Trabzon, Turkey
2. Karadeniz Technical University Faculty of Medicine, Department of Neurosurgery, Trabzon, Turkey
3. Karadeniz Technical University Faculty of Medicine, Department of Radiology, Trabzon, Turkey
4. Karadeniz Technical University Faculty of Medicine, Department of Medical Microbiology, Trabzon, Turkey
No information available.
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Summary

Idiopathic CD4 lymphocytopenia (ICL), also known as non-HIV AIDS, is a rare disease characterized by opportunistic infections, malignancies, and autoimmune diseases. Idiopathic CD4 lymphocytopenia is defined as CD4+ T-lymphocyte level below 300/mm3 in at least two measurements or less than 20% of total lymphocytes, which cannot be explained by any other condition, including HIV infection. Nocardia spp. are opportunistic microorganisms that is a frequent cause of infection in immunocompromised individuals. In this paper, we report a 50-year-old patient with ICL and intracranial brain abscess due to Nocardia farcinica and review th literature for related articles published between April 2012 and December 2018. These data suggest that there may be an underlying immunosuppression in adult patients who have infections that are recurrent, refractory, and caused by opportunistic pathogens.

Keywords:
T-lymphocytopenia, idiopathic CD4-positive, rare diseases, Nocardia, nocardiosis

References

1
Walker UA, Warnatz K. Idiopathic CD4 lymphocytopenia. Curr Opin Rheumatol. 2006;18:389-95.
2
Centers for Disease Control (CDC). Unexplained CD4+ T-lymphocyte depletion in persons without evident HIV infection--United States. MMWR Morb Mortal Wkly Rep. 1992;41:541-5.
3
Spira TJ, Jones BM, Nicholson JK, Lal RB, Rowe T, Mawle AC, Lauter CB, Shulman JA, Monson RA. Idiopathic CD4+ T-lymphocytopenia--an analysis of five patients with unexplained opportunistic infections. N Engl J Med. 1993;328:386-92.
4
Banerjee A, Biswas M, Sarkar N. Non-HIV AIDS presenting with adrenal histoplasmosis and pulmonary tuberculosis: a mesmerising case report and discussion. International Journal of Research in Medical Sciences. 2017;3:2463-7.
5
Bird A. Non-HIV AIDS: nature and strategies for its management. J Antimicrob Chemother. 1996;37(Suppl B):171-83.
6
Gholamin M, Bazi A, Abbaszadegan MR. Idiopathic lymphocytopenia. Curr Opin Hematol. 2015;22:46-52.
7
Ahmad DS, Esmadi M, Steinmann WC. Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases. Avicenna J Med. 2013;3:37-47.
8
Tania CS, David HM, Jonathan RI, Sharon CA. Nocardia Species. In: Bennett JE, Dolin R, Blaser MJ (ed). Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases. 8th edition. Elsevier Health Sciences, 2016:2853-64.
9
Ollé-Goig JE, Ramírez J, Cervera C, Miró JM. Profound reduction of CD4+ lymphocytes without HIV infection: two cases from the horn of Africa. Afr Health Sci. 2012;12:331-3.
10
Colomba C, Rubino R, Di Carlo P, Mammina C, Bonura C, Siracusa L, Titone L, Saporito L. Probable disseminated Mycobacterium abscessus subspecies bolletii infection in a patient with idiopathic CD4+ T lymphocytopenia: a case report. J Med Case Rep. 2012;6:277.
11
Rai S, Marak RS, Jain S, Dhole TN. Posterior fossa midline cryptococcoma in a patient with idiopathic CD4 lymphocytopenia. Indian J Med Microbiol. 2012;30:367-70.
12
Yashiro S, Fujino Y, Tachikawa N, Inamochi K, Oka S. Long-term control of CMV retinitis in a patient with idiopathic CD4+ T lymphocytopenia. J Infect Chemother. 2013;19:316-20.
13
Pavic I, Cekinovic D, Begovac J, Maretic T, Civljak R, Troselj-Vukic B. Cryptococcus neoformans meningoencephalitis in a patient with idiopathic CD4+ T lymphocytopenia. Coll Antropol. 2013;37:619-23.
14
Delgado-Alvarado M, Sedano MJ, Gonzalez-Quintanilla V, de Lucas EM, Polo JM, Berciano J. Progressive multifocal leukoencephalopathy and idiopathic CD4 lymphocytopenia. J Neurol Sci. 2013;327:75-9.
15
Chumpitazi BFF, Flori P, Kern JB, Brenier-Pinchart MP, Larrat S, Minet C, Bouillet L, Maubon D, Pelloux H. Severe Pneumocystis jirovecii pneumonia in an idiopathic CD4+ lymphocytopenia patient: case report and review of the literature. JMM Case Rep. 2014;1:3434.
16
Denu RA, Rush PS, Ahrens SE, Westergaard RP. Idiopathic CD4 lymphocytopenia with giant cell arteritis and pulmonary mucormycosis. Med Mycol Case Rep. 2014;6:73-5.
17
Ete T, Mondal S, Sinha D, Beyong T, Warjri SB, Pal J, Ghosh A. Idiopathic CD4 lymphocytopenia with adrenal histoplasmosis. Int J Med Sci Public Health. 2014;3:1562-4.
18
Legarth RA, Christensen M, Calum H, Katzenstein TL, Helweg-Larsen J. Cryptococcal rib osteomyelitis as primary and only symptom of idiopathic CD4 penia. Med Mycol Case Rep. 2014;4:16-8.
19
McBath A, Stafford R, Antony SJ. Idiopathic CD4 lymphopenia associated with neuroinvasive West Nile disease: Case report and review of the literature. J Infect Public Health. 2014;7:170-3.
20
Nair JP, Athavale AU, Gawande S, Shah U, Baldi M, Gupt V, Gavali V. Disseminated Cryptococcosis with Caverno-Oesophageal Fistula in a Case of Idiopathic CD4+ T-Lymphocytopenia. J Assoc Physicians India. 2014;62:66-9.
21
Sharma D, Singh N, Kaushal S, Jain S. Isolated cutaneous cryptococcosis in clinically unsuspected idiopathic CD4 lymphocytopenia. J Cytol. 2014;31:230-2.
22
Zamani A, Yosunkaya S, Reisli İ. Idiopathic CD4 T-Lymphocytopenia In A Patient With Disseminated Tuberculosis. Respir Case Rep. 2014;3:41-6.
23
Monterrubio Villar J, Perez Holgado MV, Jimenez Delgado JD, Almaraz Velarde R, Veiga Gonzalez MD. Severe colitis due to cytomegalovirus in a patient with idiopathic CD4 lymphocytopenia. Gastroenterol Hepatol. 2014;37:464-6.
24
Adjamian N, Kikam A, Wessell KR, Casselman J, Toller-Artis E, Olasokan O, Hostoffer RW. Nocardia Brain Abscess and CD4(+) Lymphocytopenia in a Previously Healthy Individual. Case Reports Immunol. 2015;2015:374956.
25
Terrell S, Shiber JR, Glassner S, Whisenant B. Cryptococcal Meningitis in a Patient with Idiopathic CD4 Lymphocytopenia. J Neuroinfect Dis. 2015;6:1-2.
26
Sikri V, Kaur H, Jain A. A rare case of idiopathic cluster of differentiation 4(+) T-cell lymphocytopenia presenting with disseminated tubercular infection. Indian J Crit Care Med. 2015;19:621-3.
27
Xia XJ, Shen H, Xu AE. Cutaneous Penicillium marneffei infection in a patient with idiopathic CD4(+) lymphocytopenia. J Dermatol. 2015;42:812-4.
28
Ish P, Singh H, Anuradha S, Dewan R. Idiopathic CD4 lymphocytopenia presenting as cryptococcal meningitis. Astrocyte. 2015;2:38-9.
29
Aotsuka Y, Uzawa A, Nishimura K, Kojima K, Yamaguchi M, Makino T, Nakamichi K, Saijo M, Kuwabara S. Progressive Multifocal Leukoencephalopathy Localized in the Cerebellum and Brainstem Associated with Idiopathic CD4+ T Lymphocytopenia. Intern Med. 2016;55:1645-7.
30
Ramalingam P, Gayathri K, Santhakumar R, Manjunath B, Karuppuswamy N, Vetriveeran B, Selvamani S, Vishnuram P, Natarajan K. Idiopathic CD4 Lymphocytopenia. J Assoc Physicians India. 2016;64:81-2.
31
Nagar VS, Kadu R, Chauhan SG, Chatterjee R, Kaushik A, Patel DV, Sood A. Clinically HIV but negative serology: Think of idiopathic CD4(+) lymphocytopenia. Indian J Crit Care Med. 2016;20:371-3.
32
Lim MS, Bermingham N, O’Broin C, Khalil A, Keohane C, Lim C. Isolated Cerebellar Spindle Cell Pseudotumor Caused by Mycobacterium avium- Intracellulare Complex in a Patient without AIDS. World Neurosurg. 2016;90:703.
33
Gupta M, Jardeleza MS, Kim I, Durand ML, Kim L, Lobo AM. Varicella Zoster Virus Necrotizing Retinitis in Two Patients with Idiopathic CD4 Lymphocytopenia. Ocul Immunol Inflamm. 2016;24:544-8.
34
Gupta HV, Gokden M, Ramakrishnaiah RH, Archer RL. Progressive multifocal leukoencephalopathy mimicking glioma in a patient with idiopathic CD4 lymphocytopenia. Acta Neurol Belg. 2016;116:391-4.
35
Malone C, Gupta ND, Kothari A, Palacios E, Neitzschman H. Radiology Case of the Month: Idiopathic CD4 Lymphocytopenia. J La State Med Soc. 2017;169:85-7.
36
Panackal AA, Rosen LB, Uzel G, Davis MJ, Hu G, Adeyemo A, Tekola-Ayele F, Lisco A, Diachok C, Kim JD, Shaw D, Sereti I, Stoddard J, Niemela J, Rosenzweig SD, Bennett JE, Williamson PR. Susceptibility to Cryptococcal Meningoencephalitis Associated With Idiopathic CD4+ Lymphopenia and Secondary Germline or Acquired Defects. Open Forum Infect Dis. 2017;4:82.
37
Sone K, Muramatsu H, Nakao M, Kagawa Y, Kurokawa R, Sato H, Niimi A. A Case of Pneumocystis Pneumonia Secondary to Idiopathic CD4+ T-lymphocytopenia: A Comparison of AIDS and Non-AIDS Patients. Intern Med. 2018;57:383-6.
38
Yagnik KJ, Skelton WP, Olson A, Trillo CA, Lascano J. A rare case of disseminated Sporothrix schenckii with bone marrow involvement in a patient with idiopathic CD4 lymphocytopenia. IDCases. 2017;9:70-2.
39
Perez-Lopez CJ, Arroyo E, Rodriguez M, Ortiz D, Nazario S. Disseminated Mycobacterium avium intracellulare leading to protein-losing enteropathy in an elderly man with idiopathic CD4 lymphocytopenia. Ann Allergy Asthma Immunol. 2017;119:390-1.
40
Jayaschandran V, Gjorgova‐Gjeorgjievski S, Siddique H. Pulmonary nocardiosis in a patient with idiopathic CD4 T‐lymphocytopenia. Respirol Case Rep. 2018;6:e00283.
41
Kano Y, Inoue H, Sakurai K, Yoshida M, Miura Y, Nakamichi K, Saijo M, Yuasa H. Idiopathic CD4-positive lymphocytopenia-associated progressive multifocal leukoencephalopathy confirmed by brain biopsy following negative results of repeated CSF-JC-virus tests: a case report. Rinsho Shinkeigaku. 2018;58:750-5.
42
Eshwara VK, Garg R, Chandrashekhar GS, Shaw T, Mukhopadhyay C. Fatal Cryptococcus gattii meningitis with negative cryptococcal antigen test in a HIV-non-infected patient. Indian J Med Microbiol. 2018;36:439-40.
43
Jundt MC, Ayalew AM, Hartman TE, Roden AC, Koo CW. Idiopathic CD4 Lymphocytopenia with Fulminant Pneumocystis jirovecii Pneumonia. Am J Respir Crit Care Med. 2019;199:35-6.
44
Gupta S, Jain SK, Kumar R, Saxena R. Idiopathic CD4 lymphocytopenia presenting with progressive disseminated histoplasmosis. Med J Armed Forces India. 2018;74:280-3.
45
Harel A, Horng S, Gustafson T, Ramineni A, Farber RS, Fabian M. Successful treatment of progressive multifocal leukoencephalopathy with recombinant interleukin-7 and maraviroc in a patient with idiopathic CD4 lymphocytopenia. J Neurovirol. 2018;24:652-5.
46
Aghoram R, Narayan SK. Progressive multifocal leukoencephalopathy in idiopathic CD4+ lymphocytopenia. J Neurovirol. 2018;24:526-8.
47
Ayub MT, Jafar MS, Khalid M, Baig MA, Mba B. Suppressed without a Cause: A Case of Idiopathic Immune Deficiency. Cureus. 2018;10:e2009.
48
Shribman S, Noyce A, Gnanapavan S, Lambourne J, Harrison T, Schon F. Cryptococcal meningitis in apparently immunocompetent patients: association with idiopathic CD4+ lymphopenia. Pract Neurol. 2018;18:166-9.
49
Nand N, Jain D, SK R, Kumar P, Verma P. Idiopathic CD4 Lymphocytopenia with Cryptococcal Meningoencephalitis: A Rare Case Report. JIACM. 2018;19:149-52.
50
Yarmohammadi H, Cunningham-Rundles C. Idiopathic CD4 lymphocytopenia. Ann Allergy Asthma Immunol. 2017;119:374-8.
51
Ambrosioni J, Lew D, Garbino J. Nocardiosis: updated clinical review and experience at a tertiary center. Infection. 2010;38:89- 97.
52
Lebeaux D, Morelon E, Suarez F, Lanternier F, Scemla A, Frange P, Mainardi JL, Lecuit M, Lortholary O. Nocardiosis in transplant recipients. Eur J Clin Microbiol Infect Dis. 2014;33:689-702.
53
Anderson M, Kuźniar TJ. Pulmonary nocardiosis in a patient with chronic obstructive pulmonary disease--case report and literature review. Pneumonol Alergol Pol. 2012;80:565-9.
54
Budzik JM, Hosseini M, Mackinnon AC Jr, Taxy JB. Disseminated Nocardia farcinica: literature review and fatal outcome in an immunocompetent patient. Surg Infect (Larchmt). 2012;13:163- 70.
55
Wilson JW. Nocardiosis: updates and clinical overview. Mayo Clin Proc. 2012;87:403-7.
56
Cheung L, Weinstein M. Idiopathic CD4 T-Cell Lymphocytopenia: A Case Report of a Young Boy With Recalcitrant Warts. J Cutan Med Surg. 2016;20:470-3.
57
Mukherjee A, Lodha R, Kabra SK. Idiopathic CD4+ T-cell lymphocytopenia. Indian J Pediatr. 2009;76:430-2.
58
Zonios D, Sheikh V, Sereti I. Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells. Arthritis Res Ther. 2012;14:222.
59
Hamidieh AA, Pourpak Z, Hamdi A, Nabavi M, Ghavamzadeh A. Successful fludarabine‐based hematopoietic stem cell transplantation in a pediatric patient with idiopathic CD4+ lymphocytopenia. Pediatr Transplant. 2013;17:109-11.
60
Alstadhaug KB, Croughs T, Henriksen S, Leboeuf C, Sereti I, Hirsch HH, Rinaldo CH. Treatment of progressive multifocal leukoencephalopathy with interleukin 7. JAMA Neurol. 2014;71:1030-5.
61
Anagnostou T, Arvanitis M, Kourkoumpetis TK, Desalermos A, Carneiro HA, Mylonakis E. Nocardiosis of the central nervous system: experience from a general hospital and review of 84 cases from the literature. Medicine (Baltimore). 2014;93:19-32.
62
Aydın M, Aksoy F, Gazioğlu G, Kaya S, Sarı A, Bayramoğlu G, Köksal İ. A Case of Brain Abscess Due to Fusobacterium nucleatum Confused with Intracranial Tumor. Türk Mikrobiyol Cem Derg. 2015;45:188- 92.
63
Minero MV, Marín M, Cercenado E, Rabadán PM, Bouza E, Muñoz P. Nocardiosis at the turn of the century. Medicine (Baltimore). 2009;88:250-61.
64
Fihman V, Berçot B, Mateo J, Losser MR, Raskine L, Riahi J, Loirat P, Pors MJ. First successful treatment of Nocardia farcinica brain abscess with moxifloxacin. J Infect. 2006;52:99-102.
65
Kandasamy J, Iqbal HJ, Cooke RP, Eldridge PR. Primary Nocardia farcinica brain abscess with secondary meningitis and ventriculitis in an immunocompetent patient, successfully treated with moxifloxacin. Acta Neurochir (Wien). 2008;150:505-6.