Lymphohistiocytosis (Hemophagocytic Lymphohistiocytosis) Questions & Answers

Updated: Mar 20, 2018
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Questions & Answers

Overview

What is hemophagocytic lymphohistiocytosis (HLH)?

What is the pathophysiology of hemophagocytic lymphohistiocytosis (HLH)?

What is the role of genetics in the pathophysiology of hemophagocytic lymphohistiocytosis (HLH)?

What is the role of Epstein-Barr virus in the pathogenesis of hemophagocytic lymphohistiocytosis (HLH)?

What are the familial forms of hemophagocytic lymphohistiocytosis (HLH)?

What is the incidence of hemophagocytic lymphohistiocytosis (HLH)?

What are the mortality rates of hemophagocytic lymphohistiocytosis (HLH)?

What are the racial predilections of hemophagocytic lymphohistiocytosis (HLH)?

How does the incidence of hemophagocytic lymphohistiocytosis (HLH) vary by sex?

How does the incidence of hemophagocytic lymphohistiocytosis (HLH) vary by age?

Presentation

What are the diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH)?

Which physical findings are characteristic of hemophagocytic lymphohistiocytosis (HLH)?

What causes hemophagocytic lymphohistiocytosis (HLH)?

DDX

What are the differential diagnoses for Lymphohistiocytosis (Hemophagocytic Lymphohistiocytosis)?

Workup

What is the role of lab studies in the diagnosis of hemophagocytic lymphohistiocytosis (HLH)?

What is the role of imaging studies are required for in the diagnosis of hemophagocytic lymphohistiocytosis (HLH)?

How is hemophagocytic lymphohistiocytosis (HLH) differentiated from other associated conditions?

What is the role of biopsy in the evaluation of hemophagocytic lymphohistiocytosis (HLH)?

Which histologic findings are characteristic of hemophagocytic lymphohistiocytosis (HLH)?

Treatment

What is included in medical care for hemophagocytic lymphohistiocytosis (HLH)?

What is the role of surgery in the treatment of hemophagocytic lymphohistiocytosis (HLH)?

Which specialist consultations are beneficial in the treatment of hemophagocytic lymphohistiocytosis (HLH)?

Medications

Which medications are used in the treatment of hemophagocytic lymphohistiocytosis (HLH)?

Which medications in the drug class Immune globulins are used in the treatment of Lymphohistiocytosis (Hemophagocytic Lymphohistiocytosis)?

Which medications in the drug class Immunosuppressant agents are used in the treatment of Lymphohistiocytosis (Hemophagocytic Lymphohistiocytosis)?

Which medications in the drug class Corticosteroids are used in the treatment of Lymphohistiocytosis (Hemophagocytic Lymphohistiocytosis)?

Which medications in the drug class Antineoplastic agents are used in the treatment of Lymphohistiocytosis (Hemophagocytic Lymphohistiocytosis)?

Follow-up

What is included in outpatient care for hemophagocytic lymphohistiocytosis (HLH)?

What are complications of hemophagocytic lymphohistiocytosis (HLH)?

What is the prognosis of hemophagocytic lymphohistiocytosis (HLH)?

What is included in patient education about hemophagocytic lymphohistiocytosis (HLH)?