(hē-mop′ă-thē) [hemo- + -pathy] Any disease of the bone marrow, blood cells, or plasma.
(hē″mō-pĕr-fū′zhŏn) [hemo-, hemato- + perfusion] The perfusion of blood through substances, such as activated charcoal or ion-exchange resins, to remove toxic materials. The blood is then returned to the patient. This technique differs from hemodialysis in that a semipermeable dialytic membrane is not used to remove toxins from the blood. SEE: hemodialysis.
(hē″mō-pĕr″ĭ-kăr′dē-ŭm) [″ + peri, around, + kardia, heart] Accumulation of blood in the pericardium.
(hē″mō-per″ĭt-ŏn-ē′ŭm, hē″măt-ō-per″ĭt-ŏn-ē′ŭm) [hemato-+ peritoneum] Bleeding into or blood in the peritoneal cavity.
(hē″mō-pek′sĭn) [hem- + pexin] A beta globulin that binds circulating hemoglobin in hemolytic diseases transporting it to the liver where it is recycled for its iron content.
(hē″mŏ-fag′ŏ-sīt″) [hemo- + phagocyte] A phagocyte that ingests red blood cells. hemophagocytic (hē″mŏ-fag″ŏ-sit′ik), adj.
ABBR: HLH. Any of several related genetic disorders characterized by over-activation of the immune system in which macrophages and histiocytes cannibalize circulating red blood cells, white cells, and platelets and produce fever, splenomegaly, jaundice, and other rashes.
INCIDENCE: The disease affects about 1 in 50,000 infants, and an uncertain number of children and adults.
CAUSES: Hemophagocytic lymphohistiocytosis is associated viral, bacterial, fungal, and parasitic infections; collagen-vascular diseases and malignancies (esp. T-cell lymphomas); and genetic and autoimmune diseases.
SYMPTOMS AND SIGNS: The most common clinical signs are fever and splenomegaly. Hepatomegaly, lymphadenopathy, jaundice, and rash (usually maculopapular) are also seen.
TREATMENT: All of the disorders are rare, and no controlled clinical therapeutic trials have been made. For some patients supportive care and treatment of underlying infection is associated with recovery in 60% to 70%.
PATIENT CARE: The care of the patient with hemophagocytic lymphohistiocytosis begins with early recognition of the syndrome, a daunting task given the severity of the illness, its resemblance to sepsis, and its multitude of potential causes. Inherited forms of the disease are treated with combinations of immune-modulating and chemotherapeutic medications. Most patients benefit from high doses of corticosteroids. Admission to an Intensive Care Unit increases the chance that multi-specialty care will be closely coordinated, e.g., between intensivists, infectious disease specialists, pathologists, and hematologists. Disease remission may require bone marrow transplantation in severely ill patients. SYN: hemophagocytic syndrome.
(hē″mō-făg″ō-sī-tō′sĭs) [″ + ″ + ″ + osis, condition] The ...