Hematological involvement in sarcoidosis: from cytopenias to lymphoma

ABSTRACT

Introduction

We present an updated overview of the hematological involvementassociated with sarcoidosis, including a management approach forcytopenias and revisiting the association with hematologicalmalignancies.

Areas covered

Theetiology of cytopenias in sarcoidosis can be attributed to two majoretiopathogenic mechanisms: infiltration of hematopoietic organs suchas the spleen and bone marrow, and autoimmune-mediated cytopenias.With respect to the association with hematological malignancies, itrequires careful evaluation of patients from a chronologicalperspective. Patients must be classified into one of three pathogenicscenarios, including preexisting hematological malignancies,synchronous development of malignancy and sarcoidosis due to commonpredisposing factors, or sarcoidosis as a predisposing factor formalignancies.

Expert Opinion

The association between sarcoidosis and hematologic involvement isbest understood as a pathogenic continuum, with cytopenias andhematologic neoplasms intertwined due to various etiopathogenicmechanisms. These mechanisms include sarcoid infiltration ofhematopoietic organs, common predisposing immunogenetics for thedevelopment of autoimmune cytopenias and malignancies, and anincreased risk of neoplasm development in patients with autoimmunecytopenias. Collaboration among the main specialties involved in theclinical management of these patients is crucial for an earlymonitoring and management.

KEYWORDS:

• Autoimmune hemolytic anemia
• immune thrombocytopenia
• leukemia
• lymphoma
• myeloma
• sarcoidosis
• sickle cell disease

Article highlights

• Existing studies on hematologic involvement in sarcoidosis are sparse and lack clinical guidelines.

• Stepwise diagnostic protocols are outlined for sarcoidosis patients presenting with cytopenias.

• Sarcoidosis rarely overlaps with genetic blood disorders but may mimic systemic autoimmune diseases.

• Therapy choices are guided by descriptive studies and focus on etiopathogenic mechanisms and symptomatic response.

• A proposed paradigm shift intertwines cytopenias and hematologic neoplasms as interconnected facets of sarcoidosis.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or material discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or mending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.