Sarcoidosis–lymphoma syndrome: a diagnostic dilemma

Abstract

Sarcoidosis and lymphoma are generally thought of as being two mutually exclusive diseases that need to be considered in the differential diagnosis of patients with hilar/mediastianal lymphadenopathy. However, there are rare patients in whom both of these diseases coexist. These patients constitute a diagnostic challenge because their presentation (ie, clinical symptoms, imaging abnormalities and even pathology) may all be atypical when each individual disease is considered separately. In this report, we describe a patient who presented with such atypical features and was eventually diagnosed as having both sarcoidosis and a B-cell lymphoma with features of splenic marginal zone lymphoma (SMZL) simultaneously. To our knowledge, this is only the second reported case of SMZL and sarcoidosis in the same patient.

Granulomatous slack skin mycosis fungoides developing simultaneously with sarcoid‐like lesions in a patient with repeated anabolic injections in the past?

“…There are a number of publications that examine the possibility of an association between sarcoidosis and lymphoproliferative diseases (LPD) (heterogeneous types of lymphoma) within the so-called sarcoid-lymphoma syndrome (Kumthekar, Raess, & Ghetie, 2019;Oskuei, Hicks, Ghaffar, & Hoffstein, 2017). In the presence of hilar/ mediastinal lymphadenopathy, both diseases should be considered in a differential diagnosis, but also the possibility of coexistence within the same patient should not be excluded (Oskuei et al, 2017).…”

“…In the presence of hilar/ mediastinal lymphadenopathy, both diseases should be considered in a differential diagnosis, but also the possibility of coexistence within the same patient should not be excluded (Oskuei et al, 2017). In this group of patients, however, it cannot always be determined which (sarcoidosis or lymphoma) is the primary disease that generates the relevant/potential pathogenetic pathways leading to the appearance of the second disorder (Oskuei et al, 2017;Papanikolaou & Sharma, 2010). Since there are often similarities in clinical symptomatology between sarcoidosis and LPD, most authors recommend a rigorous workup of these patients, including repeated lymph node biopsies, in order to achieve a precise diagnosis and avoid the risk of misdiagnosis and consequent inappropriate treatment (Oskuei et al, 2017).…”

“…There are a number of publications that examine the possibility of an association between sarcoidosis and lymphoproliferative diseases (LPD) (heterogeneous types of lymphoma) within the so-called sarcoid-lymphoma syndrome (Kumthekar, Raess, & Ghetie, 2019;Oskuei, Hicks, Ghaffar, & Hoffstein, 2017). The possibility that disturbance of tissue homeostasis by incorporation of certain adjuvants within injections (for example) in the past might have been an inducer of cutaneous T cell lymphoma and sarcoidosis/ sarcoid like lesions seems reasonable but also speculative.…”

“…The possibility that disturbance of tissue homeostasis by incorporation of certain adjuvants within injections (for example) in the past might have been an inducer of cutaneous T cell lymphoma and sarcoidosis/ sarcoid like lesions seems reasonable but also speculative. In this group of patients, however, it cannot always be determined which (sarcoidosis or lymphoma) is the primary disease that generates the relevant/potential pathogenetic pathways leading to the appearance of the second disorder (Oskuei et al, 2017;Papanikolaou & Sharma, 2010). In the presence of hilar/ mediastinal lymphadenopathy, both diseases should be considered in a differential diagnosis, but also the possibility of coexistence within the same patient should not be excluded (Oskuei et al, 2017).…”

“…There are a number of publications that examine the possibility of an association between sarcoidosis and lymphoproliferative diseases (LPD) (heterogeneous types of lymphoma) within the so-called sarcoid-lymphoma syndrome (Kumthekar, Raess, & Ghetie, 2019;Oskuei, Hicks, Ghaffar, & Hoffstein, 2017). Since there are often similarities in clinical symptomatology between sarcoidosis and LPD, most authors recommend a rigorous workup of these patients, including repeated lymph node biopsies, in order to achieve a precise diagnosis and avoid the risk of misdiagnosis and consequent inappropriate treatment (Oskuei et al, 2017). In this group of patients, however, it cannot always be determined which (sarcoidosis or lymphoma) is the primary disease that generates the relevant/potential pathogenetic pathways leading to the appearance of the second disorder (Oskuei et al, 2017;Papanikolaou & Sharma, 2010).…”