Objective: This review aims to help critical care clinicians maintain a high level of suspicion
regarding the diagnosis of Hemophagocytic Histiolymphocytosis (HLH). It describes the …

Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenopathy, most often cervical. It
has been mainly described in Asia. There are few data available on this disease in Europe. …

Limited comparative data exist for the treatment of HIV-associated non-Hodgkin lymphoma.
We analyzed pooled individual patient data for 1546 patients from 19 prospective clinical …

Objective Because it has no unique clinical, biologic, or histologic features, reactive
hemophagocytic syndrome may be difficult to distinguish from other diseases such as severe sepsis …

The homeostasis of the immune response requires tight regulation of the proliferation and
apoptosis of activated lymphocytes 1 , 2 . In humans, defects in immune homeostasis result in …

Background . Common variable immunodeficiency is characterized by recurrent infections
and defective immunoglobulin production. Methods . The DEFI French national study …

Multicentric Castleman disease (MCD) is a distinct type of lymphoproliferative disorder
associated with inflammatory symptoms and interleukin 6 (IL-6) dysregulation. In the context of …

Background Activated phosphoinositide 3-kinase δ syndrome (APDS) 2 (p110δ-activating
mutations causing senescent T cells, lymphadenopathy, and immunodeficiency [PASLI]–R1), …

X-linked lymphoproliferative syndromes (XLP) are primary immunodeficiencies characterized
by a particular vulnerability toward Epstein-Barr virus infection, frequently resulting in …

Severe ADAMTS13 deficiency occurs in 13% to 75% of thrombotic microangiopathies (TMA).
In this context, the early identification of a severe, antibody-mediated, ADAMTS13 …