WITH RECENT WHO CLASSIFICATION

1. DENDRITIC CELL TUMORS
PRESENTER : Dr. Neha Sharma
MODERATOR : Dr. Minakshi Bhardwaj

2. INTRODUCTION
• Dendritic cells (DCs) are component of innate immune system
• Most important antigen-presenting cells
• Initiate T-cell responses against protein antigens
• Have numerous fine cytoplasmic processes that resemble
dendrites, from which they derive their name

3. Background
1868 Discovery of first dendritic cells : Langerhans cells Paul Langerhans
1973 identification of DCs in mouse lymphoid tissue Steinman and Cohn
1979 skin epidermal LCs derived from bone marrow cells Katz
1990s Generation of DCs in vitro from myeloid
hematopoietic progenitors or from monocytes

4. Development Of Dendritic Cells

5. FUNCTIONS
• Antigen presentation and activation of T cells
• Inducing and maintaining immune tolerance
• Maintain immune memory in tandem with B cells

7. TYPES
DCs are found in
steady state (non-inflammatory)
non steady state (inflammatory )
• Steady state DCs include
Conventional DCs (cDCs)-
a. the myeloid-like CD11c+CD11b+CD8α- DC in the circulation and
peripheral lymphoid tissues
b. the lymphoid like CD11c+CD11b-CD8α+ DC
Type-1 interferon-producing plasmacytoid DCs (pDCs) : CD11c low
DC

8. SUBTYPES
cDCs can also be divided into
subsets according to their tissue
localizations:
• Follicular dendritic cell (FDC)
• Interdigitating cell (IDC)
• Langerhans cell (LC)
• Veiled cell (VC)
• Mucosal dendritic cell- mucosal-
associated lymphoid tissue (MALT)
K
Tonsils
Thymus
Lymphatics
Adenoids
Lymph node
Thoracic
Duct
Langerhans
cells
Interstitial
Dendritic Cells
Veiled
cells

10. LANGERHANS CELLS (LC)
• Specialized dendritic cells
in mucosal sites and skin
• Upon activation become
specialized for antigen
presentation to T cells
• Migrate to the lymph node
through lymphatics

12. INTERDIGITATING
DENDRITIC CELLS
(IDDC)
• Paracortical dendritic cells
in lymph nodes
• May be derived in part
from the Langerhans cell

13. DERMAL/INTERSTITIAL DENDRITIC CELLS
• Found in the soft tissue, dermis and most organs
• Can be increased in some inflammatory states

14. PLASMACYTOID DENDRITIC CELLS (PDC)
• Distinct lineage of dendritic cells
• The histogenetic origin - controversial
• Likely of myelomonocytic lineage
• Interferon alpha-producing PDC precursors only acquire a
dendritic appearance in cell culture
• Circulate in the peripheral blood
• Have the capacity to enter lymph nodes and tissue through high
endothelial venules

17. FOLLICULAR DENDRITIC CELLS (FDC)
• Resident within primary and
secondary B-cell follicles
• Trap and present antigen to B
cells
• Appear to be closely related to
BM stromal progenitors with
features of myofibroblasts
• Non-migrating population
• Form a stable meshwork within
the follicle

18. FIBROBLASTIC
RETICULAR CELLS
(FRC)
• Involved in maintenance
of lymphoid integrity
• Produce and transport
cytokines and other
mediators
• They ensheath the
postcapillary venules
• Express SMA

19. DCs that are not found in the steady state but develop after
infection or inflammation include
1. Monocyte-derived DCs
2. TNF-producing and inducible nitric oxide synthase (iNOS)-
expressing DCs

20. WHO Classification of Dendritic Cell Neoplasms
Tumours derived from Langerhans cells : LCH , LCS
Indeterminate dendritic cell tumour (INDCT)
Interdigitating dendritic cell sarcoma (IDDCS)
Follicular dendritic cell sarcoma (FDCS)
Inflammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma
Fibroblastic reticular cell tumour (FRCT)
Disseminated juvenile xanthogranuloma (JXG) ?
Erdheim-Chester disease (ECD) ?
Blastic plasmactyoid dendritic cell neoplasm (BPDCN)– distinct entity

21. Langerhans Cell Histiocytosis(LCH)

22. LCH TERMINOLOGIES
• Eosinophilic granuloma : Solitary lesion/unifocal
• Hand –Schuller –Christian disease : multiple lesions/multifocal
• Letterer-Siwe disease : disseminated/visceral involvement
• The term HISTIOCYTOSIS X was coined by Lichtenstein in
1953
• The term LCH was used in 1985 to cover all these syndromes

23. EPIDEMIOLOGY
• Annual incidence of LCH is 5/10,00,000 ( mostly children)
• Median age at diagnosis is 30 months old
• multisystem disease usually found in children < 2 yr old
• multifocal single site disease usually in children 2-5 yr old
• M:F-3.7:1
• 70% of children have only 1 site of disease (bone most common)
• Primary LCH of lung associated with smoking
• Association with follicular lymphoma can be seen rarely

24. PATHOGENESIS
• IL1 loop model has been proposed
• High levels of the tyrosine phosphatase SH1 in lesional tissues
• Increased levels of IL17A in peripheral blood and lesional
tissues, particularly in patients with multiorgan disease

25. CLASSIFICATION
• Eosinophilic Granuloma
• isolated bone lesion
• Hand-Schuller-Christian Syndrome
• skull lesions, exophthalmos (protruding eyes), diabetes insipidus
• Letterer-Siwe Disease
• organomegaly (enlarged liver and/or spleen), enlarged lymph nodes,
localized bone lesions, bleeding tendencies, anemia

26. LCH CLINICAL CLASSIFICATION

27. CLINICAL FEATURES
Presenting signs and symptoms varies depending upon areas
involved
• Unifocal disease :
 older children or adults
 lytic bone lesion eroding the cortex
 solitary lesions at other sites - mass lesions or enlarged lymph
nodes

28. CLINICAL FEATURES
• Unisystem Multifocal Disease
 young children
 multiple or sequential destructive bone lesions, adjacent soft
tissue masses
 Diabetes insipidus follows cranial involvement
• Multisystem Involvement
infants present with fever, cytopenias, skin ,bone lesions and
hepatosplenomegaly

29. LCH-BONES
• Presents as pain and swelling; rarely,
fracture
• Loose teeth if jaw involved
• Involved in 80% of patients
• 50% with 1 lesion; 50% with multiple
lesions
• 50% of all bone lesions found in skull and
facial bones
• 10% - vertebral body involvement
(collapsed vertebral body= “vertebra
plana”)
• Lesions in bones may last for years

30. LCH-SKIN
• Skin involved in over 50% of cases (2nd
most commonly involved organ)
• Crusty, petechial papules in scalp often
mistaken for seborrheic dermatitis
• Skin folds and diaper area frequently
involved
• Distal limbs usually spared

31. LCH-CNS
• Involvement of hypothalamic-pituitary axis (HPA) : 25% of LCH
cases
• May present with excessive thirst and large urine output,
manifestations of diabetes insipidus (permanent)
• Median age of onset : 4 years
• Seen on CT or MRI of brain
• In up to 10% of those with multisystem disease, other CNS
problems are found (degenerative CNS changes 5-15 years after
diagnosis)

32. LCH-LUNGS
• Lung involvement – 15% of LCH cases
• High-resolution CT scan : diffuse micronodular densities with cyst
formation-- honeycomb lung
• Almost never seen as the only site of involvement
• Can be asymptomatic or present with shortness of breath
• Confirmed by lung biopsy or bronchoalveolar lavage

33. LCH- LIVER/GIT
• Soft, enlarged liver often accompanied by enlarged spleen and
jaundice
• Decrease in proteins produced by liver (albumin, clotting factors)
• GI tract involvement is rarely reported but may present as
diarrhoea

34. LCH- BONE MARROW
• Hematologic problems are most often seen in those < 2 years old
• Found in patients with more severe disease
• It is characterized by anemia, neutropenia, and thrombocytopenia
• Erythrophagocytosis is a factor in the pathogenesis of anemia
• Because bone marrow and splenic involvement often occur
together, splenic sequestration further exaggerates the cytopenias

35. Lab findings
• Neutrophilia ,  ESR ,  S. Alkaline phosphatase
Biopsy
• skin, bone , liver , LN – diagnostic

36. LCH – Lymph Node

37. LCH - Lung

38. LCH- SKIN

39. LCH- SKIN

40. LCH – Bone Marrow

41. IMMUNOPHENOTYPE
POSITIVE
• CD1a
• Langerin (CD207)
• S100 protein
• Vimentin, CD68, HLA-DR
• PD-L1 (many cases)
• CD45 expression & lysozyme
content : low
• Ki-67 proliferation index :
highly variable
NEGATIVE
B-cell lineage markers
 T-cell lineage markers
(except for CD4)
CD30
Follicular dendritic cell
markers (CD1,23,35)

42. S100
CD1a Langerin

43. ULTRASTRUCTURE
• Birbeck granule :
tennis-racket
shape
200-400 nm long
and 33 nm wide
zipper-like
appearance

44. GENETIC PROFILE
• LCH has been shown to be clonal by X- linked androgen
receptor gene (HUMARA) assay
• Clonal IGH, IGK, or TR rearrangements, including some cases
with both T-cell and B-cell gene rearrangements : 30% cases
• BRAF V600E mutation : 50% cases ( no prognostic
significance)
• Somatic MAP2K1 mutations almost always occurring in BRAF
germline cases : 25% cases

45. PROGNOSIS
• The clinical course is related to staging of the disease at
presentation
• Survival for unifocal disease – 99%
• 66% mortality for young children with multisystem involvement
who do not respond promptly to therapy

46. THERAPEUTIC MODALITIES
LOCALISED
• biopsy or curettage
• radiation therapy (low dose)
• topical steroids
• intralesional steroid injections
SYSTEMIC
• oral or intravenous steroids
• oral or intravenous chemotherapy
• single agents (vinblastine, etoposide)
• combination chemotherapy

47. Targets for Therapy
• CD52 - Alemtuzumab
• Vascular Endothelial Growth Factor
• Thalidomide
• TNF-alpha antagonists
• Thymic extracts
• Suppressin – thymic hormone
• Cyclosporine
• HSCT

48. Langerhans Cell Sarcoma
(LCS)

49. LANGERHANS CELL SARCOMA
• Malignant sarcoma with Langerhans cell phenotype
• Median age : 41 years (10-72)
SITES
• Skin and underlying soft tissue – most common
• Multiorgan involvement - LN, liver, spleen, lung and bone
• High stage disease seen in 44%
• Primary nodal disease – 22%
• Hepatosplenomegaly is noted in 22%
• Pancytopenia in 11%

51. IMMUNOPHENOTYPE
POSITIVE NEGATIVE
CD1a Myeloid markers
S100 Lymphoid markers
Langerin Follicular dendritic cell markers
Ki67 : highly variable

52. S100
CD1a Langerin

53. ULTRASTRUCTURE
• Birbeck granules present
• Desmosomes and complex interdigitating cells absent

54. Genetic profile
• BRAFV600E mutation found in one case
Prognosis
• LC sarcoma is an aggressive, high-grade malignancy, with >
50% mortality from progressive disease

55. Indeterminate Dendritic Cell
Tumour
(INDCT)

56. INDETERMINATE DENDRITIC CELL TUMOUR
• Neoplasm derived from normal indeterminate cells, the
precursor of Langerhans cells
SITE
•Multiple generalized papules, nodules, or plaques on the skin
•Primary lymph node or splenic disease : rarely
CLINICAL FEATURES
• Systemic symptoms are usually not present

58. IMMUNOPHENOTYPE
POSITIVE NEGATIVE
S100 Langerin
CD1a Specific B-cell and T-cell markers
CD45, CD68, Lysozyme,
CD4 : variable
CD30
Ki-67 : variable CD163
Follicular dendritic cell markers

59. CD1a
s100

60. ULTRASTRUCTURE
• Lack Birbeck granules
• There can be complex
interdigitating cell
processes
• Desmosomes : absent

61. PROGNOSIS
• Ranges from spontaneous regression to rapid progression

62. Interdigitating Dendritic Cell
Sarcoma
(IDDCS)

63. Interdigitating Dendritic Cell Sarcoma
• Neoplastic proliferation of spindle to oval cells with phenoyptic
features similar to those of interdigitating dendritic cells
• Extemely rare neoplasm
• Site :
solitary LN involvement (most common)
Extranodal : skin & soft tissue

66. IMMUNOPHENOTYPE
POSITIVE NEGATIVE
S100 CD 21, CD35
Vimentin CD1a
Myeloid markers
Cytokeratin

67. S-100

68. ULTRASTRUCTURE
• Complex interdigitating cell processes present
• Desmosomes, birbeck granules & lysozymes absent

69. PROGNOSIS
• The clinical course is generally aggressive, with about half of all
patients dying of the disease
• Commonly affected visceral organs include the liver, spleen,
kidney and lung

70. Follicular Dendritic Cell
Sarcoma
(FDCS)

71. FOLLICULAR DENDRITIC CELL SARCOMA
• Neoplastic proliferation of spindle to oval cells showing
morphologic and phenotype features of follicular dendritic cells
• 10-20% cases associated with hyaline vascular type of
Castleman's disease

72. • Sites
• Lymph node disease in 31% of cases (mostly cervical LN)
• Extranodal disease in 58%
• Most commonly tonsil, GIT, soft tissue, mediastinum,
retroperitoneum, omentum and lung
• Both nodal and extranodal disease in 10%

73. CLINICAL FEATURES
• Patients most often present with a slow growing, painless mass
lesion
• Most patients have localized disease at presentation
• Systemic symptoms are uncommon

77. IMMUNOPHENOTYPE
POSITIVE NEGATIVE
Follicular dendritic cell marker – CD
21, CD35, CD23
Myeloid markers : CD 13,CD 33, MPO
Clusterin (strong) HMB 45
EGFR CD1a
Desmoplakin , Vimentin, Fascin
HLA-DR , EMA
CD68 and S100 : variable

78. IHC
EGFR CD21

79. ULTRASTRUCTURE
• Desmosomes and
cytoplasmic cell
processes present
• Lysozymes and Birbeck
granules absent

80. PROGNOSIS
• FDC sarcoma is usually treated by complete surgical excision,
with or without adjuvant radiotherapy or chemotherapy
• Associated with a worse prognosis
 large tumour size (> 6 cm)
 coagulative necrosis
 high mitotic count (>5 mitoses/10 HPF)
 significant cytological atypia

81. Inflammatory Pseudotumour-
like Follicular/ Fibroblastic
Dendritic Cell Sarcoma

82. • Occurs in young to middle-aged adults, with a female predilection
• Consistently associated with EBV (monoclonal episomal form)
Site
 It involves the liver or spleen or both
 Rarely, it involves the GITas a polypoidal lesion
Clinical features
 asymptomatic or abdominal distension or pain, and occasionally
systemic symptoms

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85. IMMUNOPHENOTYPE
• Positive for FDC markers, such as CD21 and CD35
• Staining ranging from extensive to very focal
• Some cases, they may be negative for FDC markers but express
SMA
• This raises the possibility of fibroblastic reticular cell
differentiation

86. CD23
EBER ISH

87. PROGNOSIS
• The tumour appears to be indolent
• Tendency to develop repeated intraabdominal recurrences over
many years

88. Fibroblastic Reticular Cell
Tumour
(FRCT)

89. FIBROBLASTIC RETICULAR CELL TUMOUR
• Very rare tumor
• Also known as CK positive interstitial reticulum cell tumours
Site
• Can occur in the lymph nodes, spleen or soft tissue

91. IMMUNOPHENOTYPE
POSITIVE NEGATIVE
SMA FDC markers (CD 21,23,35)
Desmin S100
Cytokeratin (in a dendritic
pattern)
CD68

92. ULTRASTRUCTURE
• The spindle cells show delicate cytoplasmic extensions and
features similar to those of myofibroblasts :
filaments with occasional fusiform densities
 well-developed desmosomal attachments
 rough endoplasmic reticulum
 basal lamina-like material

93. PROGNOSIS
• Available data regarding outcome are extremely limited
• The clinical outcome is variable
• Some patients die of the disease

94. Disseminated Juvenile
Xanthogranuloma (JXG)

95. Disseminated Juvenile Xanthogranuloma
(JXG)
• Characterized by a proliferation of histiocytes similar to those of
the dermal JXG
• Foamy(xanthomatous) component with Touton-type giant cells
• Originate from dermal/interstitial dendritic cell
• Most deep, visceral and disseminated forms occur by the age of
10 years
• Fifty percent within the first year of life

96. ETIOLOGY
• Known association with neurofibromatosis type 1 (NF-1)
• Patients with both are at slightly higher risk of JMML
• Patients with both LCH and JXG are also encountered

97. SITE
• Skin and soft tissues of head and neck - most common
• Disseminated forms affect mucosal surfaces – most common
upper aerodigestive tract
• Other sites - CNS, dura, pituitary stalk, eye, liver, lung, lymph
node & bone marrow

98. CLINICAL FEATURES
• Skin papules small (1-2mm) and multiple
• Optic lesions can cause glaucoma
• CNS and pituitary lesions can cause diabetes insipidus, seizures,
hydrocephalus and mental status changes
• Benign but Macrophage activation syndrome can lead to
cytopenias, liver damage and death (in the systemic forms)

101. IMMUNOPHENOTYPE
POSITIVE NEGATIVE
Vimentin, Factor XIIIA CD1a
Surface CD14 Langerin
CD68 (PGM1) - coarse granular pattern
CD163 - surface and cytoplasmic pattern
Stabilin-1 (MS-1 antigen)
Fascin (cytoplasm)
S100 (< 20% of the cases)

102. fascin
CD68
CD4
FXIIIA

103. ULTRASTRUCTURE
• Histiocytic
• Abundant cytoplasm with numerous lysosomes

104. PROGNOSIS
• All clinical forms are benign
• Multiple lesions in brain, dura or pituitary can cause local
consequences and even death
• Systemic forms that involve liver and bone marrow have been
treated with LCH-type therapy

105. Erdheim-Chester disease
(ECD)

106. ERDHEIM-CHESTER DISEASE
• Clonal systemic proliferation of foamy (xanthomatous)
histiocytes with Touton giant cells
• Rare condition
• Mean patient age 55-60 years (< 15 years can be seen)
• M:F is 3:1

107. SITE
• Virtually any organ or tissue can be infiltrated by ECD
• Skeletal (> 95%), Cardiovascular (50%), retroperitoneal (33%)
• CNS, diabetes insipidus, and/or exophthalmos in 20-30% of
patients and cerebellum involvement
• Xanthelasma of eyelids /periorbital soft tissue

108. CLINICAL FEATURES
• Bone lesions can be asymptomatic or with mild pain and
affect distal limbs
• Multisystemic disease follows an aggressive course
• Cardiovascular involvement seen by MRI or CT
 infiltration of the right atrium or AV groove with pericarditis,
effusion or tamponade
circumferential soft tissue sheathing of the thoracic and
abdominal aorta (Coated Aorta) &large arteries, leading to
renovascular hypertension

109. • Orbital infiltration (often bilateral) : exophthalmos, pain, oculomotor nerve
palsies, blindness, xanthelasma
• Pituitary gland infiltration causes diabetes insipidus,hyperprolactinaemia,
Gonadotropin insufficiency, hypotestosteronism
• CNS involvement :
Cerebellar and pyramidal syndromes
The most serious neurological complication is neurodegenerative cerebellar
disease (15- 20%)
CNS involvement is a major prognostic factor in ECD

113. IMMUNOPHENOTYPE
POSITIVE
• CD14
• CD68
• CD163
• Factor Xllla
• Fascin
• VE1 ( mutated BRAF)
NEGATIVE
• S100
• CD1a
• Langerin

114. CD163 CD68

115. GENETIC PROFILE
• Mutations in MAPK pathway genes : BRAF V600E (> 50%)
• Pl3KCA pathway gene (11% )
• NRAS mutation (4%)

116. PROGNOSIS
• The disease outcome correlates with sites of involvement
• Patients with CNS disease or multisystemic disease have a
worse outcome
• Disease activity is assessed by clinical examination, imaging and
C-reactive protein values
• ECD may respond to therapy with interferon alpha ( 5 year
survival -68%)
• Vemurafenib (inhibitor of BRAF) has recently been used with
promising results

117. Blastic Plasmacytoid
Dendritic Cell
Neoplasm(BPDCN)

118. BLASTIC PLASMACYTOID DENDRITIC CELL
NEOPLASM
• Clinically aggressive neoplasm derived from the precursors of
plasmacytoid dendritic cell
• Mean age 61-67 years
• M:F ratio is 3.3:1
• Mostly involves skin(64-100%) followed by bone marrow,
peripheral blood (60-90%) & lymph node (40-50%)

119. CLINICAL FEATURES
• Skin manifestations - most frequent
• The diagnosis is made on skin biopsy
• Three types of lesions
 isolated (one or few) purplish nodule type (two thirds of cases)
: head & lower limbs
 isolated (one or few) bruise-like papule type
 disseminated type with purplish nodules and/or papules
and/or macules : most characteristic

121. • In some patients lacking skin involvement and with leukaemic
presentation, the diagnosis is made based on peripheral blood
or bone marrow analyses
• In most cases, a fulminant leukaemic phase ultimately develops
• About 10-20% cases associated with or develop into other
myeloid disorders, most commonly CMML, MDS or AML

126. CYTOCHEMISTRY
• Negative for
Alpha-naphthyl butyrate esterase
 Naphthol AS-D choloroacetate esterase (CAE)
Myeloperoxidase

127. IMMUNOPHENOTYPING
POSITIVE
• CD4
• CD43
• CD45RA
• CD56
• PDC associated antigens - CD123 (IL3 alpha chain receptor), CD303,
TCL 1A
• TCF4
• TdT (33%)
• S-100(25-30%)
• BCL6, IRF4, and BCL2 (absent in normal PDCs)
• CD117 :Occasional cases

128. IMMUNOPHENOTYPING
NEGATIVE
• CD3
• CD13, CD16
• CD19, CD20
• Lysozyme
• MPO
• CD34 ( positive in 17% on flowcytometry)
• EBER

129. CD4
CD303
CD56
TCL1ACD123
BCL11A

130. GENETIC PROFILE
• Two thirds of patients have an abnormal karyotype
• Complex karyotypes & recurrent translocations are common
• Gene expression analysis of BPDCN reveals a unique signature
 proves that BPDCN originates from the myeloid lineage; in
particular, from resting PDCs
• TET2 - most commonly mutated gene

131. PROGNOSIS
• The clinical course is aggressive, with a median survival of
10.0-19.8 months
• Most cases (80-90%) show an initial response to multiagent
chemotherapy
• Relapses with subsequent resistance to drugs are regularly
observed have been associated with shorter survival

132. • Parameters associated with lower survival rate
High marrow or peripheral blood blastosis
low TdT expression
positivity for CD303
low Ki-67 proliferation index
CDKN2A/B deletions
mutations in DNA methylation pathway genes

133. HEMATOPOIETIC
STEM CELL (CD45+)
MESENCHYMAL
STEM CELL (CD45-)
PDC
FDC
FRC
BPDCN
FRCT :
Indolent
to
aggressive
Spindle
cells with
collagen
fibres
IHC: SMA,
Desmin;
CK, CD68
LCH:
Variable
clinical profile
LC cells
IHC: CD1a;
S100;Langerin
UM: Birbeck
granules
LCS:
aggressive
same IHC ;UM
ECD
DXG
FDCS
:mostly LN,
mod
aggressive
Spindle cells
IHC:
CD21/35/23
Clusterin;
Fascin
IDDCS : Mostly
LN; very
aggressive
Spindle cells
IHC : S100;
Fascin
Absent :CD1a,
Langerin
INDCT:
mostly localized
Oval cells
IHC:
CD1a;S100
Fascin
Absent : Langerin
Birbeck granules
–]-

134. HEMATOPOIETIC
STEM CELL (CD45+)
DERMAL/INTERSTITIAL
DENDRITIC CELL
DXG :
skin+ visceral
lesions
Foamy histiocytes
with Touton giant
cells
IHC:
Vimentin,
Factor XIIIA,
Surface CD14
CD68 ,CD163
Fascin
ECD:
Virtually any sites
Foamy histiocytes
with Touton giant
cells
IHC:
CD14
CD68
CD163
Factor Xllla
Fascin
VE1MOLECULAR
ANALYSIS

135. 123456

136. CONCLUSION
• Dendritic neoplasms are rare neoplasms that represent less
than 1% of all the neoplasms seen in the lymph nodes or soft
tissues
• Immunohistochemistry remains the mainstay of diagnosis
• Collaborative efforts are needed to better treat patients with
these rare disorders

137. CASE STUDY

138. 9 year old female presented with
• Acute chest pain of few hours
• Difficulty in breathing duration
Systemic examination revealed decreased right sided breath sounds
Other systems revealed no abnormality
There was no history of fever or hemoptysis
Thorough workup revealed no bony or skin lesions

139. INVESTIGATIONS
Investigation Result
Chest X Ray Multiple pneumatocoeles and right
sided pneumothorax
CT scan Gross distortion of architecture with
multiple thin walled cysts bilaterally
Pulmonary function test Suggestive of both restrictive and
obstructive change
Blood workup for
bacterial and viral
infections
Negative
Bronchoalveolar lavage Unremarkable

140. Thoracoscopic lung biopsy

142. S100

143. CD1a

144. FINAL DIAGNOSIS
• Pulmonary LCH

146. • NEXT ACTIVITY : Dr. Preeti
Journal club – 27/02/2019