Immunologic and inflammatory disorders/ T cell development Flashcards
——————- :a variety of proliferative disorders ‘macrophages’ (= over-production of white blood cells = histiocytes)
Histocytosis
Histiocytes can lead to ———- and ———–formation
Histiocytes can lead to organ damage and tumour formation
Is Langerhans cell histocytosis a sytemic disease?
Yes
What are langerhans cells?
type of WBCs that help the body fight infections (provide immunity)
bulidup of langerhans cells in certain body parts will lead to?
tumours or organ damage
LCH in children vs Adults
children –> acute disseminated multisytem disease
Adults –> Indolent Single organ disease
* indolnet –> slowly growing
Piutuitary invlovlement in LCH in adults is assocaited w?
Diabetes Insipidus
Calssification of langerhans cell Histocytosis
1) Acute disseminated LCH (Letterer - Siwe ds)
2) Multifocal LCH- multifocal eosinophillic granuloma ( Hand-Schuller-Christian disease)
3) Unifocal LCH - Unifocal Eosinophilic granuloma
CF of Acute disseminated LCH (Lettere- Siwe disease)
- mainly occur before the age of 2
1) Cutaneous abnormalities are present in almost 80% of patients (trunk and scalp)
2) hepatosplenomegaly
3) lyphadenopathy
4) lung involvement
5) destructive osteolytic bone lesions
In Acute disseminated LCH (Letterer - Siwe disease), when extensive BM involvement occurs pateints present w?
1) anemia, thrombocytopenia
2) recurrent infection (otitis media, mastoiditis)
3) if untreated can rapidly be fatal
(with intensive chemotherapy 50%, 5 year survival)
where are Langerhan cells found
Skin, spleen, lungs, bone marrow, liver, lymph nodes, skull
in Chronic Unifocal LCH bony lesions may cause ———?
Otitis media
CF/Epi of Multifocal LCH (Multiple Eosinophilic granuloma)
Classic HSC triad : Calvarian bone defects , exophthalmus , DI
- Epi : 2-4 yrs
* Calvarian bone : skull
CF/Epi of Unifocal LCH - unifocal eosinophilic granuloma
- Epi: age 5-15 years
1) Solitary clavarial lesions - solitary lesion of skull / vertebra /rib / mandible/ femur / ilium / scapula
2) Asymptomatic/ tenderness
3) an indolent disease (relatively benign)
* if clavarial lesion extend to the nervous system theyll cause neuro sx
which type of Histocytosis is associated w/ Tobacco smoking
Pulmonary LCH X
(Uncommon intersitital lung disease)
Epi of Pulmonary LCH X
- intersitial lung disease ass. w/ Smoking (tobacco)
- Affects young adults
- benign, RARE
CF of Pulmonary LCH X
1) Nonproductive cough (56-70%)
2) Dyspnea (40%)
3) Fatigue (30%)
4) Weight loss (20-30%)
5) Chest pain (21%)
6) Spontaneous pneumothorax, which may be recurrent, is a classic presentation found in 10-20% of patients
7) Fever (15%)
8) Cystic bone lesions (4-20%): These may be painful and may predispose the patient to pathologic fracture
what makes up a granuloma?
collection of Organized macrophages (ask Histocytes)
most common cause of lung granulomas?
fungal infections that primarily affect the lungs
Granulomas are most often the result of an ——— and most frequently occur in the ————, but can occur in other parts of the body as well
Granulomas are most often the result of an infection and most frequently occur in the
lungs, but can occur in other parts of the body as well
Apart from infections, ——- conditions can also cause granulomas
Autoimmune systemic conditions
Examples of Autoimmune systemic conditions
SLE, rheumatid arthritis, scelroderma
chracteristics of a granuloma
1) inflammation
2) fungal infections in the lungs
3) Autoimmune systemic conditions
macrophages in granulomas are often referred to as ”————-“
macrophages in granulomas are often referred to as “epithelioid”
TURE/FALSE
Loosely dispersed macrophages are not considered to be granulomas
True
they must be organized