Histiocytosis in children
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Transcript of Histiocytosis in children
Histiocytosis Histiocytosis in Childrenin Children
ByBy
Dr. Javaria RasheedDr. Javaria RasheedPostgraduate TraineePostgraduate Trainee
Paediatric Medicine Unit-IPaediatric Medicine Unit-INishtar Hospital, Multan.Nishtar Hospital, Multan.
DefinitionDefinition
Prominent proliferation or Prominent proliferation or
accumulation of cells of accumulation of cells of
monocyte-macrophage system monocyte-macrophage system
of bone marrow originof bone marrow origin..
IncidenceIncidence
1 in 200,000 children each year.1 in 200,000 children each year.
AgeAge1-15 years1-15 yearsRate peaks at 5-10 years.Rate peaks at 5-10 years.
PrevalencePrevalenceCaucasions Caucasions Affects males twice than females.Affects males twice than females.
Class 1Class 1
Langerhan’s cell histiocytosis (histiocytosis X)Langerhan’s cell histiocytosis (histiocytosis X)
Class 2Class 2Hemophagocytic lymphohistiocytoses (HLH)Hemophagocytic lymphohistiocytoses (HLH)
• Familial Hemophagocytic lymphohistiocytoses Familial Hemophagocytic lymphohistiocytoses (FHLH)(FHLH)
• Infection associated Hemophagocytic syndrome Infection associated Hemophagocytic syndrome (IAHS)(IAHS)
Class 3Class 3• Acute monocytic leukemia Acute monocytic leukemia • True malignant histiocytosisTrue malignant histiocytosis
3 Groups3 Groups 1. 1. Unifocal (eosinophilic granuloma)Unifocal (eosinophilic granuloma) Slowly progressive diseaseSlowly progressive disease Expanding proliferation of langerhan cells in Expanding proliferation of langerhan cells in
various bones, skin, lungs and stomach.various bones, skin, lungs and stomach.
2. Multifocal Unisystem2. Multifocal Unisystem Characterized by fever, bone lesions and Characterized by fever, bone lesions and
diffuse eruptions usually on scalp and in the diffuse eruptions usually on scalp and in the ear canals.ear canals.
50% of cases involve pituitary stalk leading to 50% of cases involve pituitary stalk leading to diab. insipidus.diab. insipidus.
Triad of diab. Insipidus, exophthalmos and Triad of diab. Insipidus, exophthalmos and lytic bone lesions is called Hand-Schuller lytic bone lesions is called Hand-Schuller
Christian DiseaseChristian Disease..
3 Groups3 Groups
3. Multifocal Multisystem (Letterer-Siwe 3. Multifocal Multisystem (Letterer-Siwe
Disease)Disease)
Rapidly progressive disease. Rapidly progressive disease.
Children below 2 years.Children below 2 years.
Prognosis – poor.Prognosis – poor.
5 years survival rate is only 50%.5 years survival rate is only 50%.
PathologyPathology Disease spectrum results from clonal Disease spectrum results from clonal
proliferation of proliferation of
cells resembling epidermal dendritic cells called cells resembling epidermal dendritic cells called
langerhan’s cells.langerhan’s cells.
langerhan’s cell.langerhan’s cell.Antigen presenting cell of skin.Antigen presenting cell of skin.
Hallmark of LCH.Hallmark of LCH.Clonal proliferation of cells of monocyte Clonal proliferation of cells of monocyte
lineage containing lineage containing birbeck granule birbeck granule
(Tennis Racket shaped)(Tennis Racket shaped) CDCD1a 1a positivity of lesional cells. positivity of lesional cells.
1.1. Skeleton – 80% Skeleton – 80% Skull (most common)Skull (most common) PelvisPelvis FemurFemur VertebraeVertebrae MaxillaMaxilla MandibleMandible MastoidMastoid
Clinically :Clinically :Asymptomatic, pain, local swelling, fractures, collapse Asymptomatic, pain, local swelling, fractures, collapse
of of vertebral body causing secondary compression of spinal vertebral body causing secondary compression of spinal cord, chronically draining infected ears.cord, chronically draining infected ears.
2. Skin – 50% 2. Skin – 50% Hard-to-treat scaly papular, seborrhic dermatitis Hard-to-treat scaly papular, seborrhic dermatitis
of scalp, diaper, axillary, post. auricular regions, of scalp, diaper, axillary, post. auricular regions, back, palms & soles.back, palms & soles.
Petechiae / hemorrhages. Petechiae / hemorrhages.
3. Lymphadenopathy – 33% 3. Lymphadenopathy – 33%
4. Hepatosplenomegaly – 20%4. Hepatosplenomegaly – 20%
Abdominal painAbdominal pain
5. Jaundice, Ascites.5. Jaundice, Ascites.
6. Exophthalmos – bilateral 6. Exophthalmos – bilateral
7. Gingivitis7. Gingivitis
CandidiasisCandidiasis
8. Otitis media – 30-40% 8. Otitis media – 30-40%
may lead to deafness may lead to deafness
9. 9. Pulmonary infiltration – 10-15% Pulmonary infiltration – 10-15% (on radiography)(on radiography)
10. Pneumothorax – rare 10. Pneumothorax – rare 11. Pituitary dysfunction / 11. Pituitary dysfunction /
hypothalamic involvementhypothalamic involvement Growth retardation Growth retardation Diab. insipidusDiab. insipidus
12. Primary hypothyroidism12. Primary hypothyroidism13. Systemic manifestations13. Systemic manifestations
Fever, weight loss, malaise, irritability, Fever, weight loss, malaise, irritability, FTT.FTT.
14. Anemia / Thrombocytopenia14. Anemia / Thrombocytopenia
2 Uncommon But Serious 2 Uncommon But Serious Manifestations of LCH is :Manifestations of LCH is :
1. Hepatic involvement 1. Hepatic involvement Leading toLeading to cirrhosis associated cirrhosis associated with multisystem diseasewith multisystem disease
2. CNS Involvement2. CNS InvolvementAtaxia, dysarthria, seizures and Ataxia, dysarthria, seizures and other other neurological symptomsneurological symptoms. .
DiagnosisDiagnosisTissue biopsy :Tissue biopsy :Diagnostic and easy to perform on skin or bone Diagnostic and easy to perform on skin or bone
lesions.lesions.
Others :Others :CBCCBCLFT’sLFT’sCoag. studies.Coag. studies.Skeletal SurveySkeletal SurveyChest RadiographyChest RadiographyUrine OsmolalityUrine Osmolality
Treatment & PrognosisTreatment & PrognosisSingle system disease (benign) :Single system disease (benign) : Curettage or low dose local radiation therapy.Curettage or low dose local radiation therapy. High chance of spontaneous remission.High chance of spontaneous remission. Goal is to arrest the progression of diseaseGoal is to arrest the progression of disease
Multisystem disease (Malignant) :Multisystem disease (Malignant) : Systemic chemotherapy: Vinblastine or etoposide Systemic chemotherapy: Vinblastine or etoposide
are effective in treating LCH.are effective in treating LCH. Goal is to reduce reactivation of disease and long Goal is to reduce reactivation of disease and long
term consequences. term consequences.
Experimental TherapiesExperimental Therapies
For unresponsive disease often in young For unresponsive disease often in young children with children with
multisystem disease and organ dysfunction:multisystem disease and organ dysfunction:Immunosuppressive therapy :Immunosuppressive therapy : CyclosporinCyclosporin Anti-thymocyte globulinAnti-thymocyte globulinNew agents :New agents : ImatinibImatinib 2 – Chlorodeoxyadenosine2 – Chlorodeoxyadenosine
&& Stem Cell TransplantationStem Cell Transplantation