Case Study: BCG Vaccine-Induced Tuberculosis Takes Life of a 4-Month-Old Boy with Wiskott-Aldrich Syndrome

Summary

BCG vaccination is believed to give some protection against tuberculosis, and for this reason, the Universal Immunization Program includes this vaccine. However, recently I had a very sad experience while dealing with a case of disseminated tuberculosis; better to say that the little baby’s body was just converted into packets full of AFB! In the course of investigation, we found that the 4-month-old boy was just a case of primary immunodeficiency, and he passed away 2 months after diagnosis. I think immunologists and immunization programmers could be more helpful in such cases!

Introduction

Wiskott-Aldrich syndrome is an X-linked recessive disorder which is characterized by thrombocytopenia with small platelets, eczema, recurrent infections and predisposition to autoimmune diseases and malignancy. It is a rare disorder of the immune system. It occurs in approximately 4 out of every 1 million live male births, but there is no clear racial or ethnic correlation. The purpose of this case report is to describe the spectacular inhabitation of a baby’s body by Acid-Fast Bacilli (AFB) and eventually his death, within 2 months of diagnosis. The boy was a case of Wiskott-Aldrich syndrome that we suspected, and he was diagnosed only after he had a very heavy disease.

Case Presentation and Investigations

A 4-month-old male child presented with generalized eczema, purpuric spots on pressure points, recurrent upper- and lower-respiratory tract infections, occasional bloody diarrhea, poor weight gain / malnutrition, fever and huge axillary swelling (lymphadenopathy) of 5 cm in diameter. He was given the BCG immunization vaccine on the first day of his life. His one female sibling, aged 4 years, was normal. Investigations revealed low serum IgG and IgM levels, and raised IgE and IgA levels with neutrophilia, thrombocytopenia and raised ESR. Chest X-ray was suggestive of pneumonitis but no hilar lymphadenopathy was found. Platelet count was 30 to 40 thousand per micro liter (low) with predominantly microplatelets. [s2If !is_user_logged_in()]…

[/s2If][s2If is_user_logged_in()]FNAC from the axillary swelling yielded thick pus AFB smears (Z-N stained), from which revealed numerous extracellular as well as intracellular AFB (see photomicrographs). In fact, the smears appeared red to the naked eyes, also! Soon, his condition deteriorated, and he developed multiple boils in his body and failed to respond to any kind of therapy and died.
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Differential Diagnosis

  • HIV
  • Disseminated tuberculosis
  • Lymphoma / leukemia
  • Abscesses

Discussion

A combination of multiple hematological and immunological defects and deficiencies, Wiskott-Aldrich syndrome (WAS) is linked to mutations in a gene on the short arm of the X-chromosome, which codes for the Wiskott-Aldrich syndrome protein (WASp).

In WAS, the platelets are small and malfunctioning, and they are removed by the spleen, which leads to low platelet counts. The immune deficiency is due to the inability of T-cells to become polarized and also the lower production of antibodies. This often causes more infections of the sinuses and ears.

Patients with WAS often suffer from various kinds of recurrent and severe infections and bleeding problems, which correlates with the present case. There are case reports where opportunistic infections occurred to the patients with WAS, including pneumocystis carinii pneumonia and cytomegalovirus infections; and because BCG vaccine is a live vaccine, tubercular lymphadenitis is also likely in such cases! For this reason, BCG is not recommended to SCID and HIV children. Due to T-cell dysfunction and associated deficiencies with humoral immunity, tuberculosis is also explainable in our case. The left axillary lymph node was rubbery to feel, discrete, mobile and tender, as the baby cried with gentle touch. The aspirate was sticky, thick pus. Microscopy confirmed acute suppurative inflammation, but no granuloma . Ziehl-Neelsen stained smears were red to the naked eyes, and huge numbers of intra- as well as extracellular Acid-Fast Bacilli were identified (see figures). Possible explanation to this overwhelming infection was definitely the failure in T-cell response, and thus, failure in forming a granuloma. No mediastinal or any other hematological malignancy was detected. The patient was referred to higher centres for appropriate therapy, including anti-tubercular therapy and bone marrow transplantation, if possible, but he died soon after.

Learning Points/Take Home Messages

Being an extremely rare disorder, and with multiple co-existing disorders, it is easy to miss a diagnosis of WAS. Presence of classical triads with symptoms of recurrent infections in multiple organs should raise the suspicion of WAS. Genetic testing and bone marrow transplantation are not feasible throughout countries like India. Managing complications like secondary infections may add to the patient’s quality of life. Tuberculosis and other opportunistic infections should also be suspected in such cases and must be investigated and treated accordingly. Vaccination also should be done, as per WHO guidelines!

About The Author

Dr-Swapan-Samanta-64x80
Dr. Samanta, MBBS, MD is currently a consultant at EKO Diagnostic PVT. LTD. Kolkata, India for histopath, cytopath and hematology. His other interests are: oncopathology, bone marrow and neuropathology.

 

References (click to show/hide)

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