since the advent of the hiv - aids pandemic across the globe , pneumocystis jiroveci pneumonia has been one of the most common defining illnesses of this condition .
idiopathic cd4 lymphocytopenia is one such condition where the patient presents with such aids defining illnesses but with negative serological marker for this dreaded disease . here
, we describe a case where a young man presented with pneumocystis infection but was having negative markers for hiv and thus the diagnosis of icl was concluded .
a 32-year - old adequately nourished male was admitted for complaints of shortness of breath for 1 month which had exacerbated for the past 2 days .
he used to work as a tailor with a mixed diet usually on his platter .
examination revealed a well built and nourished male with a body mass index of 25.3 kg / m who was tachypneic with the presence of tachycardia and mild fever .
oral thrush was present and scrapings were sent for fungal culture . there were no apparent risk factors for the presence of hiv or any other immunosuppressive states .
chest x - ray revealed few scattered inspiratory crackles and the saturation monitor was showing a spo2 of 87% .
high flow o2 was initiated and the patient was then shifted to the intensive care unit for further management .
given the clinical picture of the patient , pneumocystis jiroveci pneumonia ( pjp ) was high on cards , given the apparent immunosuppressed state .
empirical antibiotics , fluconazole and oral trimethoprim - sulphomethoxazole [ tmp - smx ] , was started along with steroids .
u / l ( high ) and high - resolution computed tomography of the thorax revealed ground glass opacities in both lung fields , more so being perihilar .
thus , the diagnosis of pjp was confirmed . to identify the cause of the immunosuppressed state ,
since the clinical suspicion of hiv was so high , a western blot and a hiv - rna polymerase chain reaction were ordered as well .
cd4 and cd8 counts were ordered , as a suspicion for idiopathic cd4 lymphocytopenia ( icl ) was high .
the cd8 counts were 312/mm ( normal ) with a cd4:cd8 ratio , inverted ( 0.35 ; normal : 1.41.7 ) .
he denied any history of immunosuppressive drug intake , did not experience repeated infections in the childhood , his immunoglobulin and sugar levels were normal , and the titers for htlv1 and 2 were negative .
thus , idiopathic cd4 lymphocytopenia was labeled as the cause of his immunodeficiency and was given a prophylaxis of tmp - smx and fluconazole post - treatment .
the centre for disease control in the usa in 1993 defined a new entity in the ever expanding world of immunology , known as the idiopathic cd4 lymphocytopenia .
the hiv - aids pandemic had just began to shape in those days and defining a disease , so close and yet so far from it , was a challenge that the centers for disease control and prevention had to face .
they defined this entity as the presence of cd4 counts < 300/mm or < 20% of the total lymphocyte count on more than one occasion , at least 6 weeks apart , with the absence of hiv infection or any other condition that might cause cd4 cytopenia .
icl is a heterogeneous condition which is usually diagnosed in middle age and has a slight male preponderance .
the spectrum of the presence of opportunistic infections in icl is the same when compared to a patient with hiv infection .
cryptococcosis is the most common infection associated with this disease , but candida , cytomegalovirus , nontuberculous , and tuberculous infections are common .
icl appears to be due to a physiological response where there is an altered environment of cytokines and inflammation that leads to decreased production of t - cell precursors and clonogenic capacity of the bone marrow .
this appears due to decreased interleukin-2 and increased tumor necrosis factor alpha in the internal mileu .
in addition , decreased t - cell response and increased t - cell activation have been noted .
one study had found that there is a profound defect in cxcr4 expression on cd4 cells and an abnormal intracellular accumulation of cxcr4 and its ligand cxcl12 .
loss of this cxcr4 was postulated to cause disturbance in the priming of t - cells in the secondary lymphoid organs and its improper antigen reception and subsequent activation .
barr virus , adenovirus , parvovirus b19 , and some other viral infections can cause lymphopenia in their acute stages , but it is highly unusual for them to cause an exclusive isolated cd4 cytopenia - like syndrome .
there are not any published guidelines for managing these cases , and most centers advocate using a cd4 cell count level of below 200/mm to start antimicrobial prophylaxis .
however , few case reports do suggest that icl may not be at an identical risk of infection as a patient with hiv at the same cd4 count , these data are too less to refute such claims and deny the patient of these measures . if the cd4 cytopenia is very low , i.e. below 100 and there is a presence of recalcitrant infections , then treatment with interleukin-2 is warranted ; however , this therapy has its own set of side effects and needs an informed written consent , depicting the investigational nature of the treatment protocol .
hematopoietic stem cell transplant also has shown promise in some reports to be an effective mode of treatment for this condition .
pjp is regarded as an aids - defining illness , but its presence in our patient without the serological evidence of hiv in any form leads us to investigate for this rare but important disorder .
icl is a rare disease which warrants a high index of suspicion to be diagnosed .
it can come with the same opportunistic infections as in a hiv patient , but the negative serology gives way the diagnosis .
 idiopathic cd4 + lymphocytopenia ( icl ) is a rare disorder characterized by the presence of depleted cd4 cell line without the presence of hiv infection . 
 slight male preponderance is noticed and is usually seen in the middle age group . 
 opportunistic infections are the reason for their discovery and here we describe a case where a man was diagnosed as having pneumocystis jiroveci pneumonia and oral candidiasis . 
Introduction
Case Report
Discussion
Conclusion
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