RETICULOENDOTHELIOSIS

RETICULOENDOTHELIOSIS

Tan, Candice TrixiaDDA

Reticuloendotheliosis

Affects the Reticuloendothelial

system which is characterized by

focal-tumor like collection of

histiocytes distributed in various

organs and tissues especially the

lymphoid system and bone marrow.

2 Types:

1. Non-Lipid reticuloendotheliosis or histiocytosis X

2. Lipid reticuloendotheliosis

Non-Lipid reticuloendotheliosis or histiocytosis X

primarily involves histiocytes which phagocytise physiologically normal circulating cholesterole.

abnormal proliferation of histiocytes (term for activated dendritic cells and macrophages)

a. Lettere-Siwe Diseaseb. Hand-Schuller-Christian Diseasec. Eosinophilic Granuloma

A. Lettere-Siwe Disease

A rare and fatal genetic disorder considered to be a type of histiocytosis where histiocytes proliferate in the body.

The disorder is believed to be inherited in an autosomal recessive pattern

Caused by excessive proliferation of histiocytes.

It is most commonly seen in children less than two years old.

A. Lettere-Siwe Disease

Known as Malignant Histiocytosis.

Most serious form of Histiocytosis X.

Poor prognosis during first period of life

If left untreated, there will be an Overgrowth of bone marrow.

Bone lesion present with illdefined radioluscencies.

Characteristics:

Soft tissue accumulation of histiocytes

Hepatosplenomegally: spleen & liver enlargement

Lymph-adenopathy Fever Anemia Skin rash Cachexia

oDeath usually occurs in a few weeks or months.

Dental Features:• Diffused alveolar bone loss• Exfoliation of deciduous teeth

 Treatment:

• Chemotherapy for multiorgan involvement

• Surgery or radiation therapy for single bone involvement

B. Hand-Schuller-Christian Disease

A progressive disease beginning in childhood that is marked by protruding eyeballs, diabetes insipidus, and a softening and destruction of bone, especially the skull, and is caused by abnormal cholesterol metabolism.

This systemic disorder classically involves the flat bones of the skull, ribs, pelvis, scapula, or a combination.

Occurs first decade of life.

Cranial lesions are always present in this disease

Dental Features: “floating teeth”

Gum swelling and necrosis

Tooth loss caused by apical and gingival infiltration is common in older patients.

The teeth appears in x-ray as floating space with bone destruction extending beyond original limit of periodontal ligaments.

Histologically: Shows characteristics sheath

of histiocytes and variable number of eosinophils.

Lipidization of histiocytes. Increase collagenisation

Treatment: Involves irradiation of the

bone lesions, very potent medications to prevent further bone and lung damage and conventional management of the diabetes insipidus.

Characteristics: Infiltration of pituitary

hypothalamic and retro-orbital areas.

Bone lesion ( cranial lesions are always present in this disease)

Exopthalmus (caused by orbital tumor mass)

Diabetes insipidus Liver, spleen and

lymphnodes may be affected.

C. Eosinophilic Granuloma

includes a group of disorders characterized by a variety of tumor like lesions, which arise from from clonal proliferation of histiocytes.

Occurs second or third decade of life. Common in children less than 12 years of age (but can occur in young adults.

More common to males

sites of involvement: skull (most common), mandible

hallmark is presence of an osseous lesion most frequently arising in skull and femur; osseous lesions may produce rapidly destructive bone lesions.

occurs as a result of metabolic defects in the reticuloendothelial system

Charecteristics:

Lesions appear in marrow spaces

Lesions of flat bone appears punched out; long bones become irregular, lobulated and expansive. (radiographs)

Dental Features: Sore mouth Fetid breath Pus Pain Swelling of gingiva Retarded healing of

extraction wounds Histopathologic

Appearance: Presence of sheaths of

histiocytes and eosinophils with occasional areas of necrosis.

Treatment: Antibiotics breathing assistance with a

respirator physical therapy hormone replacement to deal

with hormonal dysfunction

Lipid reticuloendotheliosis

a. Neimann, Pick Disease b. Gaucher’s Diseasec. Amaurotic Familial Idiocy (Tay-Sacks Disease)d. Metachromatic Leukodystrophye. Fabry’s Diseasef. Neurovisceral Lipidosisg. Farber’s Lipogranulomatosis 

*Disease with oral lesions.

*

B. Gaucher’s Disease

an inherited metabolic disorder that leads to the accumulation of a particular fatty substance throughout the body.

 results when an individual inherits defective gene for the enzyme glucocerebrosidase from both parents. Gaucher's disease is common in its more mild forms, especially among Jewish persons of Eastern European origin.

May occur in adult or infantile form

Lungs and brain are affected

More marked in the mandible

Characteristics: Hepatosplenomegall

y Weakness Skin pigmentation Bone lesions Pancytopenia

(reduction in the formed elements of the blood)

Dental Features: Irregular osteoporotic defects

with gradual expansion Thinning of cortex and resorption

of roots in teeth. Hemorrhage during extraction

Treatment: Spleen removal Bone marrow transplants Purification of adequate

quantities of alglucerase (glucocerebrosidase-beta-glucosidase) has allowed intravenous administration on a regular basis. The drug reduces the body's total supply of glycolipid and improves symptoms.