A Multirefractory Lymphomatoid Granulomatosis Successfully Treated with Gemcitabine as a Single Agent: Case Report and Review of the Literature

Case Report

Ann Hematol Oncol. 2021; 8(4): 1338.

A Multirefractory Lymphomatoid Granulomatosis Successfully Treated with Gemcitabine as a Single Agent: Case Report and Review of the Literature

Ku J¹*, Bron D¹, Wind RD², Meuleman N¹ and Massaro F¹

¹Department of Hematology, Bordet Institute, Universite Libre de Bruxelles, Belgium

²Department of Anatomopathology, Bordet Institute, Universite Libre de Bruxelles, Belgium

*Corresponding author: Ku JK, Department of Hematology, Bordet Institute, Boulevard de Waterloo 121, 1000 Bruxelles, Belgium

Received: February 22, 2021; Accepted: March 22, 2021; Published: March 29, 2021


Lymphomatoid Granulomatosis (LYG) is a rare, EBV-driven disease with angiocentric and angiodestructive pleomorphic lymphocytic infiltrates. Pulmonary involvement is frequent and responsible for pulmonary manifestations which may be associated with systemic symptoms. Its rarity makes it difficult to diagnose, and the diagnosis is usually made after several months of investigation. There is no consensus on treatment, which can range from a waitand- see approach to multidrug therapy with allogeneic hematopoietic stem cell transplantation, according to histological grade. The lack of consensus does not help in the management of these patients. We report the case of a patient with a multirefractory LYG, who achieved a Complete Remission (CR) with gemcitabine as single agent salvage treatment.

Keywords: Lymphomatoid granulomatosis; Non Hodgkin lymphoma; Rare lymphoma; Lymphoproliferative disorder; Autologous stem cell transplantation


LYG was first described by Liebow AA in 1972 [1]. It was introduced into the WHO classification of hematological tumors and lymphoid tissues in 2001 and the 2016 revision describes it as a mature B-cell neoplasm [2]. It is a very rare entity, counting for <1% of non Hodgkin’s lymphoma, with approximately 600 cases reported in the literature, which affects men more than women (H:F ratio >2:1), in adulthood with a mean age of 48 years and is related to Epstein-Barr Virus (EBV) and therefore more likely to affect immunocompromised subjects, such as patient suffering from HIV, congenital immunodeficiency or with hematopoietic stem cell transplantation. Pulmonary involvement is cardinal, being present in approximately 90-100 % of cases [1-5].

Morphological examination reveals an angiocentric and angiodestructive polymorphic lymphoid infiltrate. Granulomatosis is an inconstant finding. The histological grade depends on the degree of EBV expression assessed by In Situ Hybridization (ISH). A higher expression determines a higher grade, correlating with disease aggressivity. It is especially important to distinguish grade 1-2 (<5 EBER+/hpf, 5-20 EBER+/hpf) from high grade, grade 3 (> 50 EBER+/ hpf) as this will determine different therapeutic approach [2].

In immunophenotypic study, atypical B cells mark CD20, CD30 may be positive, but CD15 is negative. Monoclonality can be demonstrated in high grades by immunoglobulin chains rearrangement [2,4]. Because of the very rare occurrence of the entity, there is no consensus concerning standard treatment: experts recommendations are primarily based on data from case reports and retrospective series [3].

Clinical Case

Our patient is a 60-year-old Caucasian man, who consulted for chest pain associated with hemoptysis, an involuntary weight loss of 20kg and asthenia. The patient is a construction worker presenting several comorbidities: arterial hypertension, type 2 diabetes mellitus, chronic obstructive gold III pulmonary disease and thromboembolic disease without an identified contributing factor treated 2 years earlier. Family history was unremarkable. His treatment consisted of an ACE inhibitor, a beta blocker and an oral hypoglycemic agent. Clinical examination reveals a decreased vesicular murmur on the right lung field at pulmonary auscultation.

A Computed Tomography (CT) of the chest shows an excavated mass in the upper right lobe of 7×4×5 cm with a central bell of 4×4×2 cm, areas of postero-basal lower right lobe consolidation and a centimetric right hilar adenomegaly, as well as a left paramediastinal mass of 6×6×6 cm at the level of the esogastric junction. The 18F-FDG - Positron Emission Tomography (PET) CT-scan assessment reveals hypermetabolic areas in the voluminous right lung mass (SUV max 15.12), pulmonary nodules (SUV max 13.09), necrotic-looking masses at the level of the diaphragmatic pillars (SUV max 11.23) and several hypermetabolic hepatic lesions (SUV max 14) (Figure 1).

Citation: Ku J, Bron D, Wind RD, Meuleman N and Massaro F. A Multirefractory Lymphomatoid Granulomatosis Successfully Treated with Gemcitabine as a Single Agent: Case Report and Review of the Literature. Ann Hematol Oncol. 2021; 8(4): 1338.