Histiocytosis Questions & Answers

Updated: Nov 08, 2018
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Questions & Answers

Overview

What is Langerhans cell histiocytosis (LCH)?

What is the pathology of histiocytosis disorders?

What is the pathology of Langerhans cell histiocytosis (LCH)?

What is the global prevalence of Langerhans cell histiocytosis (LCH)?

What is the prognosis of polyostotic sclerosing histiocytosis?

What are the sexual predilections of histiocytosis?

Which age groups have the highest prevalence of Langerhans cell histiocytosis (LCH)?

How is risk determined for Langerhans cell histiocytosis (LCH)?

Presentation

Which clinical history findings are characteristic of Langerhans cell histiocytosis (LCH)?

How are histiocytosis disorders classified?

Which histiocytosis disorders are classified as C group (non–Langerhans cell histiocytosis of skin and mucosa)?

What are the subtypes of cutaneous non–Langerhans cell histiocytosis with a major systemic component?

Which histiocytosis disorders are classified as L (Langerhans) group diseases?

What are the subtypes of Langerhans cell histiocytosis (LCH)?

What are the subtypes of polyostotic sclerosing histiocytosis (Erdheim-Chester disease [ECD])?

What are the subtypes of cutaneous non–Langerhans cell histiocytosis?

Which histiocytosis disorders are classified as M Group (malignant histiocytosis)?

Which histiocytosis disorders are classified as R Group (Rosai-Dorfman disease)?

Which histiocytosis disorders are classified as H Group (hemophagocytic lymphohistiocytosis)?

What is the WHO classification of histiocytic disorders?

What is the Histiocyte Society classification of histiocytosis syndromes?

Which histiocytosis classifications are no longer used?

What is juvenile xanthogranuloma (JXG)?

What is sinus hyperplasia?

What is primary hemophagocytic lymphohistiocytosis (HLH)?

What is Griscelli syndrome type II?

What is HLH reactive hemophagocytic syndrome?

What is histiocytic necrotizing lymphadenitis?

Which physical findings are characteristic of Langerhans cell histiocytosis (LCH)?

What causes histiocytosis?

What causes hemophagocytic lymphohistiocytosis (HLH)?

What causes polyostotic sclerosing histiocytosis (Erdheim-Chester disease)?

What causes Langerhans cell histiocytosis (LCH)?

DDX

Which conditions are included in the differential diagnoses of Langerhans cell histiocytosis (LCH)?

Which conditions are included in the differential diagnoses of Langerhans cell histiocytosis (LCH) with bone involvement?

Which conditions are included in the differential diagnoses of Langerhans cell histiocytosis (LCH) with skin lesions?

Which lymphatic disorders are included in the differential diagnoses of Langerhans cell histiocytosis (LCH)?

Which conditions are included in the differential diagnoses of Langerhans cell histiocytosis (LCH) with otorrhea?

Which conditions are included in the differential diagnoses of multisystemic Langerhans cell histiocytosis (LCH)?

Which conditions are included in the differential diagnoses of Langerhans cell histiocytosis (LCH) with hypothalamic and pituitary lesions?

Which infectious conditions are included in the differential diagnoses of histiocytosis?

What are the differential diagnoses for Histiocytosis?

Workup

What is the role of lab tests in the workup of histiocytosis?

Which lab tests and imaging studies are performed in the long-term monitoring of Langerhans cell histiocytosis (LCH)?

How is polyostotic sclerosing histiocytosis diagnosed?

What is the role of imaging studies in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of pulmonary function tests in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of biopsy in the workup of Langerhans cell histiocytosis (LCH)?

Which histologic findings are characteristics of Langerhans cell histiocytosis (LCH)?

Treatment

How is Langerhans cell histiocytosis (LCH) treated?

How is polyostotic sclerosing histiocytosis treated?

Which specialist consultations are beneficial to patients with Langerhans cell histiocytosis (LCH)?

Why is long-term monitoring required in patients with Langerhans cell histiocytosis (LCH)?

Medications

What is the role of medications in the treatment of Langerhans cell histiocytosis (LCH)?

Which medications are used as monotherapy in the treatment of Langerhans cell histiocytosis (LCH)?

What is the role of chemotherapy in the treatment of Langerhans cell histiocytosis (LCH)?

How is recurrent or refractory Langerhans cell histiocytosis (LCH) treated?

Follow-up

Which patients should receive long-term monitoring of Langerhans cell histiocytosis (LCH)?

What are the possible complications of Langerhans cell histiocytosis (LCH)?

What is the prognosis of Langerhans cell histiocytosis (LCH)?

What is included in patient education about histiocytosis?