Primary adult-onset macrophage activation syndrome with multisystemic tissue phagocytosis

International Journal of Hematology
Anastasios AndreopoulosGeorge Vaiopoulos

Abstract

The histiocyte disorders are divided into the following 3 categories according to the specific lineage of the histiocytes involved and their biological behavior: the dendritic cell-related disorders, which include Langerhans cell histiocytosis and dermal dendrocyte disorders; the macrophage cell disorders, hemophagocytic lymphohistiocytosis being the main entity; and the malignant histiocyte disorders. We present a case of a 36-year-old woman who was referred to our hospital because of fever of unknown origin, lethargy, anemia, and impaired hepatic function. Following a thorough investigation, we diagnosed extensive histiocyte-mediated phagocytosis in many areas (skin, liver, bone marrow), without any identifiable cause. The disease was controlled by immunosuppressive therapy, and the patient remains in complete remission. This case supports the concept of idiopathic generalized histiocyte activation as a distinct entity; this putative disease entity produces massive phagocytosis, regardless of the type of histiocyte differentiation. Similar cases necessitate further study for classification and management.

References

Mar 1, 1991·Annals of Internal Medicine·K F WongS Y Ha
Apr 30, 1998·Hematology/oncology Clinics of North America·J I HenterG Janka
Oct 24, 2000·Leukemia & Lymphoma·S ImashukuF Kawano
Nov 15, 2000·Emerging Infectious Diseases·D N Fisman
Jul 9, 2004·International Journal of Hematology·Tsuyoshi Muta, Yujiro Yamano
Apr 2, 2005·Acta Haematologica·Shinji KounamiNorishige Yoshikawa

❮ Previous
Next ❯

Citations

Jun 13, 2015·Scandinavian Journal of Clinical and Laboratory Investigation·Meir Djaldetti, Hanna Bessler

❮ Previous
Next ❯

Related Concepts

Related Feeds

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.

Birth Defects

Birth defects encompass structural and functional alterations that occur during embryonic or fetal development and are present since birth. The cause may be genetic, environmental or unknown and can result in physical and/or mental impairment. Here is the latest research on birth defects.