Case No. :	115
Month :	July
Year :	2008
Contributor :	Dr. Prashant Naphade

Other Cases

Case Report: A three–year-old boy presented with distension of abdomen, reduced appetite, low grade fever since four months and jaundice since 15 days. There is no significant family history and the developmental milestones were normal. On examination, the patient was febrile, icteric and pale. He had tachycardia and was tachypneic. The abdomen was distended with hepatomegaly. Air entry was normal on both sides and the heart sounds were normal.

LABORATORY FINDINGS:

Hb- 9.8 gm%, WBC- 24,000/cumm,Platelets- 400,000 /cu mm
ESR- 58 mm/hr, Total bilirubin- 7.8 mg/dl, Direct bilirubin- 2.9 mg/dl
SGOT- 107units/L, SGPT- 67units/L

RADIOLOGICAL FINDINGS:

A frontal chest radiograph of the chest shows subtle nodular opacities. Right hilar and right paratracheal lymph enlargement is seen. Hepatomegaly and mild spelnomegaly are apparent.

Fig. 1

USGabdomen revealed massive hepatomegaly (liver span 17 cms) and mild splenomegaly ( 9cm) .Liver showed coarse heterogeneous echotexture and multiple echogenic and hypoechoic nodules and cystic lesions which show posterior acoustic enhancement.

Periportal and paraaortic lymphadenopathy was noted.

Fig. 2	Fig. 3
Fig. 4	Fig. 5

CT abdomen shows hepato-splenomegaly with multiple, well-defined hypodense nodules and cystic lesions scattered in both lobes of the liver. The nodules showed subtle peripheral enhancement on post contrast scan. Extensive periportal and paraaortic lymphadenopathy was noted which showed homogenous enhancement on contrast injection. There was no free fluid in the abdomen..

Fig. 6	Fig. 7
Fig. 8	Fig. 9

CT Thorax revealed multiple well defined cystic lesions scattered throughout the lung fields. Subcarinal and posterior mediastinal lymphadenopathy noted.

Fig. 10	Fig.11

10 days later, the child developed severe respiratory distress, the chest radiograph was repeated and it shows bilateral pneumothorax with underlying collapse of the lungs.

Fig. 12

ICD was inserted in both the pleural cavities and 5 days later repeat radiograph of the chest revealed good lung expansion.

In the meanwhile, bone marrow biopsy was performed and revealed normocellular marrow with trilineage haemopoeisis; blast cells were not increased.

An isotope bone scan did not reveal any abnormality.

USG guided Liver biopsy was performed after correcting the coagulopathy.

The histopathologic features were consistent with Langerhan’s cell histiocytosis.

Immunohistochemistry study reveals CD1a & S-100 positivity and negative for CD34.

DIAGNOSIS:

Final diagnosis of Langerhans cell histiocytosis was made.

The child is being treated with weekly doses of IV Vinblastine ( 2 mg diluted in 10cc NS) for 6 weeks and Prednisolone ( 20 mg/day) in tapering doses over 2 weeks.

DISCUSSION:

Langerhans cell histiocytosis is characterized by the proliferation of specialized bone marrow–derived Langerhans cells and mature eosinophils.

The working group of the Histiocyte Society has divided histocytic disorders into three different groups: (1) dendritic cell histiocytosis, (2) erythrophagocytic macrophage disorders, and (3) malignant histiocytosis now known as T-cell lymphoma

Langerhans cell histiocytosis belongs to the group of dendritic cell histiocytosis.

AGE: The disease can occur in any age group. The peak incidence is in 1-5 yrs.

SEX: Male predominance 2:1

CLINICAL PRESENTATION: The clinical presentation of LCH is dependent on the extent of dissemination of the disease.

• Unifocal LCH is characterized by the development of solitary bony lesions at any site. These are often asymptomatic
• Multifocal disease presents with osteolytic lesions and organ dysfunction to varying extent. Patients with Hand-Schüller-Christian syndrome often present with recurrent episodes of otitis media and mastoiditis or with polyuria and polydipsia.
• Letterer-Siwe disease presents with symptoms suggestive of a systemic infection or malignancy with a generalized skin eruption, anemia, and hepatosplenomegaly.

CAUSES : The etiology of LCH is unknown.

• LC proliferation may be induced by a viral infection, a defect in intercellular communication (T cell–macrophage interaction), and/or a cytokine-driven process
• Cigarette smoking may play a role as a chronic irritant in the development of eosinophilic granuloma of the lung.

IMAGING FINDINGS:

Skeletal system:

1. Skull: most common site. “ Beveled edge”, rounded and lytic lesions with sharp and serrated margins & absence of sclerosis in the calvarial location is the most common pattern of involvement. CT shows greater destruction of the outer table with a soft tissue mass associated with calcification in some cases.
2. Ribs: Second most common site, often multiple lesions- destruction associated with expansion of the bone.
3. Pelvis & Scapula: bone destruction and minimal periosteal reaction is noted.
4. Spine: Thoracic spine is involved commonly with lytic vertebral body destruction, compression fractures & vertebra plana.
5. Tubular bones: rare below knees & elbow.

Thorax:

1. Lungs: Alveolar pattern in the early stage, destructive pulmonary changes, thin walled pulmonary cysts, hyperexpansion, ground glass opacities and recurrent pneumothorax.
2. Mediastinum: Lymphadenopathy, cavitationg mediastinal mass, thymic enlargement, anterior mediastinal mass with punctuate calcification.

Liver: Hepatomegaly, Nodules which appear hyperechioc on ultrasound, hypodense on CT, hyperintense on T1 & hypointense on T2. Diffuse cystic lesions can be present with periportal fibrosis and sclerosing cholangitis.

GIT: coarse nodular small bowel mucosa, dilatation and thickening of mucosal folds, mesenteric lymphadenopathy and ascitis.

CNS: communicating hydrocephalus, hypothalamic- pituitary axis involvement, peripheral hemispheric lesions with a dural attachment, brainstem involvement

TREATMENT:

• For single system disease : The options available to treat the bony lesions are either local curettage & excision or with indomethacin or a short course of systemic steroids. Radiation therapy is reserved for large painful lesions at inaccessible sites.
• For multisystem disease : Combination of cytotoxic drugs and systemic steroids is used.