Histiocytoses

Question 1

In Langerhan’s cell histiocytosis, Hematopoietic, liver, lung, or spleen involvement confers 35-55% mortality even with treatment

Answer 1

True

Question 2

T/F

Eruptive xanthomas need to be distinguished from xanthoma disseminatum or other non-Langerhans cell histiocytosis

Answer 2

True
Eruptive xanthomas is a benign condition associated with hypertriglyceridaemia
xanthoma disseminatum is also benign and the lesions look almost identical but it is a type of non-Langerhans cell histiocytosis derived from dermal dendrocytes

Question 3

What are the 4 major variants of Langerhan’s cell histiocytosis?

Answer 3

LangEr HAN’s C(s)ell

• Letterer-Siwe disease
• Eosinophilic granuloma
• Hand-Schuller-Christian disease
• Congenital self-healing reticulohistiocytosis (CSHR)

Question 4

What are the 3 key factors in prognosis of Langerhan’s cell histiocytosis?

Answer 4

1. Age (poor if under 2 years old)
2. Extent of disease
3. Presence of vital organ failure
   (lung, liver, bone marrow in children; lung and liver in adults)
   NB. Hematopoietic, liver, lung, or spleen involvement 35-55% mortality even with treatment

Question 5

What are the clinical features of Langerhan’s cell histiocytosis?

Answer 5

Skin - seb derm like scalp rash, papular, nodular, pustules/vesicles or purpuric rash, eroded flexures, erythema, yellow-brown scale
LNs - lymphadenopathy, may be erosive w/ sinus formation
BM - splenomegally, pancytopenia, Low IgA + IgG
GI/Liver - hepatomegally, ascites, diarrhoea
Lungs - infiltrates esp in smokers
Bones - fracture or bone pain
Ears - chronic otitis media
Eyes - exopthalmos - late sign in HSC variant
Dental - periodontal involvement
Endocrine - diabetes insipidus (due to pituitary infiltration)

Question 6

What are the investigations for Langerhan’s cell histiocytosis?

Answer 6

Biopsy to confirm diagnosis - Reniform coffee bean nuclei cells, Positive for S100, CD1a, and Langerin
Full systems rw and examination
FBC and ESR for bone marrow and haematological involvement
ELFTs for liver involvement
EPP for BM (Ig changes)
Serum and urine osmolarity for DI
Skeletal survey for bone involvement (multiple X-rays of major bones)
CXR for lung involvement+/- HRCT of lungs, Liver USS, MRI brain, BMbiopsy

Question 7

What is the treatment for Langerhan’s cell histiocytosis?

Answer 7

Refer to haematologist +/- paeds, ortho, resp, endo, dentist
Treatment depends on system involved
Mild single-system skin disease;
TCS
Topical antimicrobials or Antibiotics
nbUVB, PUVA
top nitrogen mustard
thalidomide
Multi-system disease – chemotherapy

Question 8

What is Rosai-Dorfman disease?

Answer 8

‘Sinus Histiocytosis with massive Lymphadenopathy’
rare type of non-Langerhan’s cell, Non-dendritic cell histiocytosis
affects age 10-30, M>F, all races
>600 reported cases
- Massive, painless cervical lymphadenopathy
- Fever
- Mild anemia and neutrophilia, high ESR
- 40% have extranodal involvement with skin being most common site (bone, CNS, genitourinary, GIT, heart, thyroid)
Skin involved in 10%; can be only extranodal site involved + rare skin-limited form
Multiple red-brown or xanthomatous macules, papules, nodules or plaques of eyelids and malar regions
Often indolent, self-limiting
Many lesions are asymptomatic and heal spontaneously Rare fatal cases
Poor prognosis if; disseminated LN Dx or involvement of liver, kidneys or lungs