Pneumocystis jerovecii pneumonia in a patient with untreated chronic lymphocytic leukaemia: a novel case and postulations concerning the mechanism

Abstract

SUMMARYChronic lymphocytic leukaemia (CLL), even when severe, is not directly associated with opportunistic infections. Opportunistic infections that occur with CLL are almost exclusively related to immunosuppression caused by chemotherapeutic drugs used to treat CLL. We report a case of Pneumocystis jirovecii (PJ) pneumonia that occurred in a patient with untreated CLL with pulmonary involvement. We suspect that PJ pneumonia resulted from an inadequate immune response in the lung parenchyma resulting from excessive local accumulation of CLL cells. BACKGROUND

Atypical Pneumocystis jirovecii pneumonia in previously untreated patients with CLL on single-agent ibrutinib

“…Four of 5 cases occurred in previously untreated patients receiving ibrutinib in first-line and presented as mild disease, varying from asymptomatic multifocal pulmonary infiltrates to chronic cough. Atypical presentations of PCP have been described, 7,12 but it is unclear if mild disease reflects the natural history of PCP in this patient population or increased vigilance and early identification.…”

“…The risk of infection appears to be highest in the first 6 months and decreases thereafter, consistent with indications of improving immune function on ibrutinib. 6 Although Pneumocystis jirovecii pneumonia (PCP) has been rarely reported in previously untreated CLL, 7,8 treatment with a fludarabine-based regimen has long been thought to increase the risk of PCP 9 and prophylaxis is recommended for patients treated with chemoimmunotherapy. 3,4,10 Here we report, for the first time, 5 cases of PCP in 96 CLL patients treated with singleagent ibrutinib under 2 prospective studies.…”

Pneumocystis jirovecii Pneumonia in a Treatment-Naive Patient With Chronic Lymphocytic Leukemia

“…It has been postulated that parenchymal infiltration with CLL cells interferes mechanically with local immune responses contributing to susceptibility. In the prior case of PCP in CLL, transbronchial needle aspiration demonstrated airway mucosa infiltrated with CLL [4]. Our patient’s BAL cytology had atypical lymphocytes consistent with CLL, but he did not have a transbronchial biopsy.…”

“…Because the risk of Pneumocystis jiroveci pneumonia (PCP) is increased while receiving therapy for CLL prophylaxis is the standard of care. Infection with Pneumocystis jiroveci ( P. jirovecii ) has been identified in patients receiving all of the aforementioned treatment regimens, however, it has only once previously been described in a CLL patient who was treatment naïve [4]. We report a case of PCP in a patient with CLL who had received no prior treatment.…”

Histoplasmosis diseminada como manifestación inicial del linfoma linfocítico de células pequeñas

“…Las bacterias encapsuladas y los virus respiratorios, como el de la influenza, son los más prevalentes en los pacientes que no han sido diagnosticados y, por lo tanto, no se han tratado (4). Las infecciones por gérmenes oportunistas son menos frecuentes, incluso en estadios avanzados de la enfermedad (5).…”