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Serial opportunistic infections after ruxolitinib treatment in primary myelofibrosis patient

1연세대학교 원주의과대학 내과, ²연세대학교 원주의과대학 감염내과, ³연세대학교 원주의과대학 병리과, ⁴연세대학교 원주의과대학 혈액종양내과

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Introduction Ruxolitinib is an oral inhibitor of Janus Kinase approved for the treatment of primary myelofibrosis (PMF). Since it is not specific to mutant JAK2, it involves down-regulating proinflammatory cytokines. We report this ruxolitinib treated PMF patient who had serial several opportunistic in- fections (hepatitis B virus (HBV) reactivation, bacterial pneumonia (suspicious), pneumocystis jirovecii pneumonia (PJP), and invasive aspergillus pneu- monia) within a month, and experienced severe dyspnea after ruxolitinib discontinuation which is hard to differentiate between ruxolitinib withdrawal syn- drome and PJP. Case report In May 2018, 68-year-old man hospitalized with a fever. He had been taking ruxolitinib for 3 years since JAK2V61F mutation positive essential thrombocytosis (ET) progressed to PMF. Initial AST/ALT was 117/164 U/L, and quantitative HBV DNA level was 17692.8 copy/mL.

The computed tomography (CT) scan revealed consolidative lesion on the left lower lung field (LLL). Reactivation of HBV and bacterial pneumonia was considered. Tenofovir and anti-pseudomonas antibiotics were initiated, and ruxolitib was discontinued. Two days later, he started to complain about dyspnea. LLL consolidation was aggravated in chest PA, and teicoplanin was added. On hospital day (HD) 8, his O2 saturation down to 79%. Chest image showed ground glass opacity in both lungs. Under the impression of Ruxolitinib withdrawal syndrome or PJP pneumonia, steroid and trimethoprim-sulfa- methoxazole (TMP/SMX) were initiated. Later, we found out PJP PCR was positive. He discharged on HD17. Three weeks after steroid and TMP/SMX, we found multiple new nodular lesions in both lungs. Needle aspiration was performed, and it revealed fungal hyphae compatible with invasive aspergillosis. Voriconazole was initiated, then nodular lesions were improved in 2 weeks. Ruxolitinib was reinstituted. Now he is taking ruxolitinib, tenofo- vir, and voriconazole with no symptom at all. Conclusion A physician should be alert for opportunistic infection durings ruxolitinib treatment, and monitor for ruxolitinib withdrawal syndrome carefully after ruxolitinib discontinuation

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Primary Cardiac non-Hodgkin lymphoma: the role of early Surgical Biopsy

가톨릭대학교 의과대학 서울성모병원 혈액내과

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A 41-year-old man was being followed up for 1st degree AV block with PSVT since 2010. On the 4th year of the regular follow-up, the echocardiogram study discovered hypertrophic myocardium in the LV. The Cardiac MRI delineated the characteristics of the ventricular wall thickening, appearing as slightly hyper-intense on T2-weighted imaging (Figure 1a). In 2015, the patient was admitted due to sustained VT. Increased glucose uptakes in the LV wall (SUV 7.4) and RV wall (SUV 3.9) were noted in the PET-CT, suggesting either inflammatory disease or neoplasm (Figure 1b). To identify the lesion, percu- taneous transvenous biopsy (PTVB) was performed, but only revealed chronic inflammation with lymphoid aggregation. In 2017, the patients were admit- ted again due to dyspnea. Echocardiogram verified heart failure with substantial amount of pericardial effusion. Since malignant lymphoma was highly sus- pected in further images, we decided to perform a more aggressive diagnostic method-surgical thoracoscopic excisional biopsy. Histopathology confirmed a primary mediastinal large B cell lymphoma, with positive for CD20 and negative for CD3 in immunohistochemistry staining. The patient was treated with standard R-CHOP chemotherapy. The response was dramatic; he achieved CR after the 3rd cycle, and maintained CR after the end of the 8th cycle. The cardiovascular symptoms were much relieved. Cardiac masses are often found incidentally, but sometimes accompany with cardiovascular symptoms. It is important to determine whether the mass is a normal variant, imaging artifact, thrombus, or tumor. Echocardiography is suited to be the initial imaging mo- dality because of the wide availability, lack of radiation, and relatively low cost. The gold standard cardiac imaging technique to distinguish tumor from thrombus is cardiac MRI, but the final diagnosis of tumor should be done with biopsy. As it is difficult to perform biopsy of a primary cardiac tumor, there are limitations that can be relied on primarily imaging studies. Therefore, even if negative results are found by less invasive biopsy methods, we recom- mend determining an active surgical biopsy quickly.