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case_images: PET/CT and BM examination. (A) PET/CT scanning before chemotherapy showed no signs of tumor. (B) Many hemophagocytes and (C) abnormal lymphocytes were observed in the 6th BM smear (H&E stain, 400x). (D) The 6th BM biopsy (H&E stain, 400x) showed intravascular (endothelial cells; blue arrows) aggregation of clustered or cord-like medium-sized atypical cells (red arrows). Immunohistochemical staining was positive for CD20, CD79a, BCL-6, and Mum-1 and negative for CD3, CD10, and EBER1/2-ISH. (E) The last BM examination, which was conducted on December 13, 2019, revealed no abnormal cell infiltration. BM, bone marrow; H&E, hematoxylin and eosin.
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case_images: A positron emission tomography/computed tomography (PET/CT) scan revealed minor bilateral pleural and pericardial effusion and mild splenomegaly without significant hypermetabolism ( Figure 1A ). Enhanced magnetic resonance imaging (MRI) of the brain showed no obvious abnormalities ( Supplementary Figure 1 ). Consequently, the sixth BM examination, which was conducted on December 6, 2016, revealed a large number of hemophagocytes and intravascular aggregation of medium-sized atypical lymphocytes ( Figures 1B-D ) that were positive for CD20, CD79a, BCL-6, and Mum-1 but negative for CD3, CD10, and EBER1/2-ISH, ultimately resulting in a diagnosis of IVLBCL. The latest BM examination, which was conducted on December 13, 2019, revealed no abnormal cell infiltration ( Figure 1E ).
case_images: Treatment timeline. The patient presented with recurrent fever, limb edema, hypoalbuminemia, hypotension and cytopenia in July 2016. His inflammatory markers were slightly elevated while TB-IGRA yielded positive results. Therefore, infectious diseases were first considered. Empirical broad-spectrum antibiotic therapy and diagnostic antituberculosis therapy were administered and led to little efficacy.Subsequent laboratory findings confirmed the diagnosis of HLH. Glucocorticoids were given to mitigate inflammation. The 4th BM examination in October 2016 yielded a positive bacterial culture for Staphylococcus hemolyticus and vancomycin was administered. Subsequently, localized edema developed into anasarca. CLS was established. In addition to the ED regimen for HLH, treatment with hydroxyethyl starch-based artificial colloid was initiated to treat CLS. In December 2016, numerous telangiectasias occurred on the erythematous skin. The 6th BM examination confirmed the diagnosis of IVLBCL. Immunochemotherapy with R-CHOP was administered and resulted in prompt alleviation of symptoms, signs and laboratory abnormalities. During hospitalization, the patient received multiple albumin infusions and diuretic therapy. A total of 7 cycles of R-CHOP followed by ASCT were administered, and PET/CT scanning revealed no signs of tumor during mid-treatment or before ASCT. At the time of this publication, he had maintained disease-free survival for more than 6 years. *The patient was treated with cefoperazone/sulbactam, imipenem/cilastatin, levofloxacin and moxifloxacin for anti-infection. #Including isoniazid, levofloxacin, ethambutol, and pyrazinamide. ALB, serum albumin; BM, bone marrow; BP, blood pressure; TB-IGRA: tuberculosis interferon-gamma release assay; CRP: c-reactive protein; PCT: procalcitonin; HLH, hemophagocytic lymphohistiocytosis; CLS, capillary leak syndrome; IVLBCL, intravascular large B-cell lymphoma; ED, etoposide and dexamethasone; R-CHOP, rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone; ASCT, autologous peripheral stem-cell transplantation.
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