Updated: 5/2/2020

Langerhans Cell Histiocytoses

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Snapshot
  • A 12-year-old boy presents to the emergency room for pain in in his right forearm with minimal trauma while playing tag. Radiography shows a fracture of the radius. A skeletal survey also shows 2 other lytic bone lesions on the cranium. He is referred to the pediatric oncologist for further workup and possible biopsy for a Langerhans cell histiocytosis.
Introduction
  • Overview
    • Langerhans cell histiocytosis (LCH) describes a group of conditions caused by proliferation of dendritic (Langerhans) cells
      • traditionally classified by 4 subtypes, but more recent classification is based on extent of dissemination
 
Langerhans Cell Histiocytosis (LCH)

Letterer-Siwe Hand-Schuller-Christian Hashimoto-Pritzker (Congenital Self-Healing Reticulohistiocytosis)
Eosinophilic Granuloma
Demographics
  • < 2 years of age
  • 2-6 years of age
  • Neonatal period
  • 7-12 years of age
  • Pulmonary involvement seen in ages 30-40 years
Involvement
  • Skin, bone, and viscera
    • pulmonary infiltrates, lymph nodules, liver, and spleen
  • Skin, bone, and viscera
    • liver, spleen, and lymph nodes
  • Skin-limited
  • Primarily bone > skin
  • Pulmonary involvemen
Prognosis
  • Poor prognosis with disseminated disease
  • Chronic and progressive
  • Rapid spontaneous resolution is likely
  • Good prognosis
 
  • Epidemiology
    • incidence
      • rare
    • demographics
      • males > females
    • risk factor     
      • smoking
        • pulmonary involvement
  • Pathogenesis
    • mechanism
      • proliferation of Langerhans cells, which are immature and are unable to stimulate T-cells via antigen presentation
        • thought to be reactive or neoplastic
      • BRAF V600E mutation can be found in LCH
  • Prognosis
    • worse with BRAF V600E mutation
    • worse if there’s failure to respond to 6 weeks of treatment
Presentation
 
Clinical Presentation of LCH 

Letterer-Siwe Hand-Schuller-Christian Hashimoto-Pritzker (Congenital Self-Healing Reticulohistiocytosis)
Eosinophilic Granuloma
Symptoms
  • Acutely disseminated lesions affecting the skin, bone, and internal organs
  • Fever
  • Bone pain
  • Triad of diabetes insipidus, osteolytic bone lesions (cranium), and exophthalmos
  • Skin lesions that crust and resolves spontaneously
  • Solitary indolent bone lesions (cranium most common) with spontaneous bone fractures
Physical exam
  • Pink papules, pustules, vesicles, petechiae, purpura, and erosions on the scalp, flexural surfaces, and trunk
  • Secondary impetiginization common
  • Skin findings similar to Letterer-Siwe
  • Multipe red to brown papulonodules, some with erosions
  • Tenderness at site of bone lesions
 
  • Other symptoms
    • recurrent ear infections
    • dizziness
    • headache
    • limping (due to leg pain)
    • failure to thrive
    • developmental delay
Imaging
  • Bone radiograph
    • views
      • cranium or other skeletal survey
    • findings
      • punched out lytic lesions
  • Chest radiograph
    • findings
      • may show pulmonary nodules or infiltrate
Studies
  • Serum studies
    • liver function test
      • to evaluate for liver involvement
    • baseline complete blood count
  • Urine studies
    • to evaluate for diabetes insipidus
  • Pulmonary function test
    • o evaluate for pulmonary involvement
  • Histology
    • S100+, CD1a+, and Langerin+
    • proliferation of histiocytes with mixed inflammatory infiltrate
  • Electron microscopy
    • Birbeck granules or “tennis rackets"
Differential
  • Multiple myeloma
    • key distinguishing factors
      • may also have “punched out” lytic bone lesions
      • typically affects older adults, and protein electrophoresis of the serum (SPEP) will reveal monoclonal gammopathy
Treatment
  • Medical
    • corticosteroids
      • indications
        • to slow autoimmune reaction
    • chemotherapy
      • indications
        • for severe cases in children
      • drugs
        • cyclophosphamide, etoposide, methotrexate, and vinblastine
    • radiation therapy
      • indications
        • bone lesions
    • vasopressin
      • indications
        • diabetes insipidus
    • bisphosphonates
      • indication
        • prevent bone destruction from bone lesion
  • Surgical
    • curettage or excision
      • indications
        • bone lesions
Complications
  • Bone fractures
  • Spontaneous pneumothorax
  • Infections
  • Hearing impairment
 

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Questions (2)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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