Bilateral cervical lymphadenopathy and pancytopenia

Bilateral cervical lymphadenopathy and pancytopenia

Dr Ira Shah.
Medical Sciences Department, Pediatric Oncall, Mumbai.

Medical Sciences Department, Pediatric Oncall, Mumbai
Clinical Problem
A 5 years old girl born of third degree consanguineous marriage presented with bilateral swelling over the neck since 18 days which is gradually increasing in size and associated with pain in swallowing. She has fever since 10 days and cough since 3 days. She is full term normal delivery and immunized till date. She was treated for the same with IV antibiotics as acute follicular tonsillitis but had no response. On examination, she bilateral cervical adenopathy 4 cm x 4 cm tender and firm. Tonsils were enlarged and congested. Pallor was present. On systemic examination, she had hepatomegaly. She was investigated for the cervical lymphadenopathy.
• Hemoglobin = 10.2 gm/dl
• WBC count = 9,400/cumm [52% polymorphs, 48% lymphocytes]
• Platelet count = 1,91,000/cumm
• ESR = 60 mm at end of 1 hour
• X-Ray Chest = Normal
• Mantoux test HIV = Negative
• Lymph node FNAC = Reactive lymphadenitis
• CMV IgM & Toxoplasma IgM = Negative
• Paul Bunnel test = Negative

She was treated with IV crystalline Penicillin for 10 days but there was no relief in fever and size of lymph nodes remained the same.
Peripheral smear showed atypical lymphocytes and liver & renal function tests were normal.
USG Abdomen showed hepatosplenomegaly with multiple lymph nodes. On Day 10 of presentation, a CBC showed
• Hemoglobin = 7.4 gm/dl
• WBC count = 3,500/cumm [58% polymorphs, 40% lymphocytes, 2% eosinophils]
• Platelet count = 81,000/cumm
• ESR = 14 mm at end of 1 hour

What is the cause of the cervical lymphadenopathy ?
Expert’s opinion:- Dr. Ira Shah:
This child has presented with a large bilateral cervical lymphadenopathy. Hence one may consider a differential diagnosis of
1. Infections : HIV, CMV, Toxoplasma, Rubella, EBV, Tuberculosis, and Brucella
2. Malignancy : Lymphoma
3. Histiocytosis
4. Macrophage activation syndrome
5. Others such as sarcoidosis, Kikuchi’s disease

In this child, there was no evidence of TB, Toxoplasma, CMV, HIV. Also she had no rash ruling out rubella. Thus one would consider the other possibilities. Since this child developed pancytopenia during the course of the disease, one would consider Lymphoma, Macrophage activation syndrome and histiocytosis.
This child’s serum cholesterol, triglycerides, fibrinogen, Prothrombin Time (PT) and Partial Thromboplastin Time (PTT) were normal which would be otherwise abnormal in Histiocytosis. However ESR is a clue. With MAS, the ESR will decrease whereas with malignancy ESR would rise. Thus in this child one may consider a diagnosis of MAS.
The lymph node biopsy in the child showed Langerhans histiocytosis. Serum Ferritin in this child was elevated suggestive of Macrophage activation syndrome.

MAS is characterised by high fever, hepatosplenomegaly, lymphadenopathy, pancytopenia, liver dysfunction, disseminated intravascular coagulation, hypofibrinogenemia, hyperferritinemia, and hypertriglyceridemia. Despite marked systemic inflammation, the erythrocyte sedimentation rate (ESR) is paradoxically depressed, caused by low fibrinogen levels.
Compliance with ethical standards
Funding:  None  
Conflict of Interest:  None
Cite this article as:
Shah I. Bilateral cervical adenopathy and pancytopenia. Pediatr Oncall J. 2007;4: 45.
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