
Periorbital tumor of Langerhans histiocytosis. Via Wikipedia. Original from University of Cincinnati. CC BY 2.5.
Contents
- 1 Definition
- 2 Occurrence
- 3 Symptoms
- 4 Diagnosis
- 5 1. The "L" (Langerhans) group
- 6 2. The “C” group (Cutan (skin) and mucocutane (skin and mucous membranes)
- 7 3. “M” (Malignant / Cancer) Group. Malignant histiocytosis
- 8 4. "R" (Rosai-Dorfman) group and various forms that do not attack skin
- 9 5. “H” Group. Hemophagocytic lymphohistiocytosis (HLH) and Macrophage activation syndrome (MAS)
- 10 Literature
Definition
Histiocytosis is a group of rare diseases there histiocytes (special white blood cells: tissue macrophages) by mistake are stored in large quantities in various organs. Histiocytosis was previously (WHO 1987) divided into three main groups:
- Langerhans histiocytosis
- Non-Langerhans
- Malignant (malignant) histiocytosis
Later, it is proposed to be divided into five main groups (reference: Emile JF, 2016).
- Older names of the diseases are Histiocytosis-X, Letter-Seven disease, Hand-Chüller-Christian disease and diffuse reticulo-endotheliosis
- Tissue samples (biopsy) are sometimes described by histiocytosis as "eosinophilic granuloma"
Occurrence
Histiocytosis is defined as one rare disease.
Symptoms
Symptoms and findings vary.
- The most common disease onset is in childhood and early adolescence,
- Some types can debut among adults
- Erdheim-Chester's disease
- Pulmonary histiocytosis (subgroup of Langerhans cell hypertrophy, attacks lungs)
- Reticulo-histiocytosis (may resemble Arthritis, RA)
Diagnosis
The diagnosis is made on the basis of symptoms and investigation findings. One uses CT-MR and PET / CT examinations, but the result of tissue test (biopsy) is crucial.
1. The "L" (Langerhans) group
-
Langerhans cell histiocytosis
Langerhans Histiocytosis Swelling of the head / scalp. Failla V, Rare Tumors (2010). Open
- Symptoms and severity vary from harmless to severe
- 76% are children under the age of 10
- Fever
- Weight Loss
- Multiple skeletal swelling, most often in the head / skull
- Eczema and wounds, most often on the head (80%)
- Hepatosplenomegaly (large liver and spleen) and lymph node enlargements
- Lungs with asymptomatic, nodular opacities (pulmonary histiocytosis X)
-
Erdheim-Chester disease
-
Erdheim-Chester, histiocytosis with skeletal changes close to the ankle joint. Adawi M, Mdicine (Baltimore) 2016, CC BY-NC-ND 4.0
- Multi-organ Illness
- Men more frequent than women
- Debut age on average 55 years (16-80year)
- Skeletal sclerosis in the long bones
- Please read more on our own page about Erdheim-Cehster's disease here
-
Juvenile xanthogranuloma with manifestation beyond the skin
- Skin abnormalities
- Nervous system (CNS), liver and spleen, eye, throat, muscles
- Most often children under the age of 1, but can also be seen in older children and adults
2. The “C” group (Cutan (skin) and mucocutane (skin and mucous membranes)
-
Reticulo-histiocytosis (restricted and multisentric type); please read more here
- Multisentric type
- Severe arthritis (joint inflammation) in hands
- Widespread skin and significant Arthritis-changes
- Usually women 50-60 years
- Varying, swinging disease activity over several years
- Multisentric type
- Xantogranulom group
- Different forms that can attack from young children to adults
- Some or many red-yellow nodules in the skin
- 0,5-1,0 cm in diameter
- If other organs are also included, the disease is classified in the “L” Group (see above)
-
Sea-blue histiocytosis
- Skin affection with histiocytes in adipose tissue
- Hereditary or after prolonged parenteral fatty nutrition
3. “M” (Malignant / Cancer) Group. Malignant histiocytosis
- Symptoms: Cough, decreased appetite, anemia, dyspnoea
- Lungs, lymph nodes, liver, spleen and CNS may be affected by histiocytic infiltration (biopsy)
- Various primary forms that are separated from lymphoma by a tissue sample
- Secondary forms of lymphoma and leukemia
- Subtype of acute Myeloid leukemia
- More than 20% blasts at bone marrow examination
Acute monocyte leukemia
4. "R" (Rosai-Dorfman) group and various forms that do not attack skin
- Rosai Dorfman Disease, Sinus Histiocytosis with Massive Lymphadenopathy (SHML)
- Definition
- Rare disease with many different manifestations including swollen lymph
- The tissue sample (biopsy) that is critical to the diagnosis shows a typical image of histiocytes containing lymphocytes.
- Usually attacking children and young adults
- Symptoms
- Fever, night sweats, fatigue, weight loss
- Medical investigation and findings
- Varying symptoms and changes: Swollen lymph nodes, most often on the throat
- Skin changes with fat loss
- Stuffy nose and sinuses
- Pressure behind eyes and double vision
- Enlarged spleen
- Osteonecrosis
- Diagnosis
- Symptoms and investigation findings may cause suspicion
- Biopsy is essential
- Differential diagnosis
- GPA (Wegener's granulomatosis)
- Panniculitis
- Langerhans histiocytosis
- Lymphoma
- Sarcoidosis
- Tuberkulosis (Tbc)
- IgG4 related disease
- Treatment
- Immunosuppressive drugs
- Corticosteroids may be sufficient in some
5. “H” Group. Hemophagocytic Lymphohistiocytosis (HLH) og Macrophage activation syndrome (MAS)
- Cytokin storm with uncontrolled activation of lymphocytes and macrophages (special types of white blood cells in the immune system)
- See Macrophage activation syndrome (MAS)
Literature
- Emile JF, 2016
- Allen CE, 2015 (Treatment)
- Grischikowsky M, 2015 (Management)
- Grans Compendium in Rheumatology