Mastocytosis

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Mastocytosis
Body Parts: Whole Body
Medical Subjects: Skin
Overview

What Is Mastocytosis

What disease is mastocytosis?

Mastocytosis (MC), also known as mastocytosis, is a disease spectrum composed of multiple clinical manifestations, with abnormal infiltration and accumulation of mast cells in one or more organs as the main manifestation, often involving the skin, digestive tract, bone marrow, liver and spleen, and lymph nodes, manifested as itchy skin, nausea, vomiting, diarrhea, abdominal pain, etc.

At present, there is no effective treatment. The current treatment is mainly to block the release of mediators and avoid various causes for the release of mast cell mediators.

The mild cases may have no obvious clinical symptoms, while the severe cases have a high degree of invasion and poor prognosis. Isolation is not required during periods of illness, and although the majority of patients remain unchanged in life span, their quality of life is severely compromised.

Is mastocytosis common?

The disease can occur at any age and its prevalence is currently unknown.

What are the types of mastocytosis?

The WHO classifies them into the following subtypes:

Cutaneous mastocytosis:

  •  
  • Maculopapulous cutaneous mastocytosis/urticaria pigmentosa and some rare type: maculopapulous skin MC, patchy skin MC, nodular skin MC, disseminated skin MC, telangiectatic skin MC;
  • Diffuse cutaneous mastocytosis;
  • Mast cell tumor of skin.

Inert systemic mastocytosis:

  • Smoldering systemic mastocytosis;

  • Mastocytosis with isolated bone marrow involvement;

  • Systemic mastocytosis complicated with associated clonal non-mast cell lineage hematological disorders;

  • Aggressive systemic mastocytosis;

  • Mast cell leukemia;

  • Mast cell sarcoma;

  • Extra-cutaneous mast cell tumor.

Cause

What Is The Cause Of Mastocytosis

What reason is mastocytosis caused?

The etiology remains unclear and studies have suggested a possible association with gene mutations, but further studies are needed.

Is mastocytosis contagious?

No.

Symptom

What Symptom Does Mastocytosis Have

What are the common manifestations of mastocytosis?

All subtypes have similar clinical manifestations, and the skin, gastrointestinal tract, lymph nodes, liver and spleen, and bone marrow are the common involved organs:

  • Skin involvement: The most common cutaneous manifestation is pigmented urticaria, which presents as small yellowish or reddish-brown macules or slightly raised papules. The rash of diffuse cutaneous mastocytosis is diffuse in distribution and usually manifests as yellowish-brown skin hypertrophy and may have an orange-peel appearance. Mast cell tumor is a variant of skin pigmented urticaria. Mast cell sarcoma is a very rare disease that can develop in distant transmission and can also be a stage of leukemia.

  • Gastrointestinal symptoms: more common to. They presented with abdominal pain, diarrhea, nausea and vomiting, and our patient had a high incidence of peptic ulcer disease.

  • Musculoskeletal pain: associated with osteopenia or osteoporosis.

  • Bone marrow: about one-third of patients have an associated hematological disorder.

  • Liver and spleen involvement: liver and spleen deficiency, abnormal liver function, etc.

How does mastocytosis develop?

According to the different development of pathological classification, dermatological prognosis is the best, followed by inert systemic, both of which often progress gradually and can last for decades.

Systemic mastocytosis complicated by hematological disorders progresses more rapidly and in a complex manner.

Mast cell leukemia is rare and progresses rapidly. Mast cell sarcomas are extremely rare.

Detect

How To Check For Hypermastocytosis

Under what circumstances should we consider the possibility of mastocytosis?

It should be considered in adults if one or more of the following symptoms occur:

  • Unexplained ulcers or malabsorption;

  • Imaging examination revealed abnormal bone scans, hepatosplenomegaly and lymphadenopathy in the patient;

  • Abnormal peripheral blood;

  • Immediate allergic reaction;

  • Or there is unexplained hypotension.

How is mastocytosis diagnosed?

In diagnosing this disease, physicians rely mainly on clinical manifestations, histopathology, and laboratory tests, which are then classified into different subtypes.

What check does mastocytosis need to do?

  • Hematology Lab: Including routine blood test, liver and kidney function, blood biochemistry, immunology, etc.

  • Tissue biopsy: Includes skin biopsies, bone marrow aspiration biopsies, and biopsies of tissues from other sites for definitive diagnosis.

Prevention

How To Prevent Mastocytosis

Can mastocytosis be prevented? How?

The etiology of this disease is unknown, and there are no clear preventive measures.

How does the patient of hypertrophy cell increase disease prevent to appear complication?

Early detection, early diagnosis, early treatment, regular review, and timely treatment of discomfort.

Treatment

How To Treat Mastocytosis

Which branch should mastocytosis see?

Dermatology, gastroenterology, hematology or rheumatology.

Can mastocytosis heal itself?

No!

How is mastocytosis treated?

There is currently no effective treatment and although the life expectancy of most patients remains unchanged, their quality of life is seriously impaired. Therefore, the current drug therapy is mainly to block the production and release of mediators and avoid various precipitating factors for the release of mast cell mediators. Specific treatments include:

  • Skin pruritus and flushing: H1 and H2 antihistamines are commonly used, and leukotriene inhibitor or sodium cromoglycate may be added.

  • Adrenaline injection: used to treat hypotension and allergic reaction. H1 antihistamines can be administered prophylactically to patients with recurrent hypotension; Oral psoralen combined with A-band ultraviolet (UVA) exposure therapy is used to treat skin mast cell hyperplasia, and local glucocorticoid can be used to treat skin lesions.

  • Gastrointestinal symptoms: inhibition of gastric acid secretion.

  • Osteoporosis and fracture: patients with mild osteoporosis can be supplemented with calcium and bisphosphate; For invasive diseases, drugs such as interferon can be used.

  • Hematological system: In addition to the use of interferon, etc., targeted therapy can be administered.

Does mastocytosis need to be hospitalized?

Need, generally cannot heal itself.

Can mastocytosis effect a radical cure?

No!

Life

What Should Mast Cell Increase Disease Patient Notice In Life

What should be noticed in the life and diet of patients with mastocytosis?

Should avoid a variety of precipitating factors caused by the release of mast cell media, such as physical environment stimulation, emotional stress, infection, vaccines, etc., pay attention to a healthy light diet, avoid spicy excitant food.

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