HLH Masquerading Lymphoma: Diagnostic Dilemma and Treatment Outcomes
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ORIGINAL ARTICLE
HLH Masquerading Lymphoma: Diagnostic Dilemma and Treatment Outcomes Megha Verma1
•
Nitin Gupta1 • Jasmita Dass2 • Jyoti Kotwal2 • Ajay Sharma1
Received: 13 September 2019 / Accepted: 31 December 2019 Ó Indian Society of Hematology and Blood Transfusion 2020
Abstract A case series to illustrate difficulties faced in diagnosis, management and subsequent therapeutic approach patients presenting with HLH secondary to lymphoma. A retrospective review of patients treated for HLH and lymphoma in Clinical Hematology department of a tertiary care hospital in North India, was performed from Jan 2017 to April 2019. Follow up was included till September 2019. Diagnosis of HLH was made using HLH 2004 criteria along with H score. Only patients who fulfilled HLH 2004 criteria were included. Nine patients were treated during above period, three patients with Hodgkins lymphoma, two patients had DLBCL and four patients had T-cell lymphoma. All patients presented with features of HLH and underlying lymphoma was detected on further evaluation. All patients had H score above the cut off value for diagnosis of HLH. Out of 9 patients, 6 received lymphoma directed chemotherapy and 1 was given only steroids, 1 received IVIG with steroids. 1 died early, before institution of therapy. Out of the 6 patients who received chemotherapy, all attained remission status but two patients had early relapse. In the remaining 3 patients who could not be started on chemotherapy, all died within 3 weeks of presentation. Underlying lymphoreticular malignancy should be actively searched in adult patients presenting with HLH. Early diagnosis and initiation of disease specific therapy with or without specific HLH directed treatment can improve the historical poor prognosis.
& Megha Verma [email protected] 1
Department of Clinical Hematology and Bone Marrow/Stem Cell Transplant, Sir Ganga Ram Hospital, New Delhi, India
2
Department of Hematology, Sir Ganga Ram Hospital, New Delhi, India
Keywords HLH Lymphoma LAHS Hemophagocytosis
Introduction Hemophagocytic lymphohistiocytosis (HLH) is an immune regulatory disorder characterized by clinical signs and symptoms of extreme inflammation and development of cytopenias [1]. Initially described as a disease of pediatric population with familial inheritance, through time, syndrome of HLH has been recognised in adults as well [1]. HLH is caused by a defect in inflammatory signals that results in uncontrolled hypercytokinemia, usually in a setting of congenital or acquired defective natural killer (NK)/T-cell function in the cytotoxic pathway. Even with currently recommended therapy, HLH is frequently fatal. Early institution of therapy is critical to control the hypercytokinemia that otherwise will lead to end-organ failure and death. HLH has been traditionally divided into primary and secondary [1]. Primary HLH typically manifests in children with documented genetic abnormalities of the cytotoxic function of NK cells and T cells. Secondary HLH tends to occur at older ages in the set
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