KEY CLINICAL MESSAGE:
Clinicians should consider the possible association of Daunorubicin with
SJS, administer it with caution and promptly evaluate all subsequently
developing cutaneous reactions with high index of suspicion for
Steven-Johnson Syndrome.
CASE REPORT :
A 2 years old male child diagnosed with Precursor B cell Acute
Lymphoblastic Leukaemia/Lymphoma (ALL) underwent a multi-drug regimen
remission induction chemotherapy as per the modified
Berlin-Frankfurt-Münster (BFM) 90 ALL protocol with Vincristine
1.4mg/m2/dose intravenously once a week (D1,8), Prednisolone 40mg/m2/day
orally daily (D1-8), L-Asparaginase 6000Units/m2/dose intravenously
q.a.d from D2 (a total of 3 doses), Methotrexate 8mg/dose intrathecally
once a week (D1,8) and Daunorubicin 30mg/m2/dose intraveneously on D8.
About two weeks after the initiation of first induction chemotherapy,
the patient developed cutaneous erythema around the face extending to
the chest with ulceration of mucosal surfaces of oropharynx which
quickly progressed into confluent erythematous and necrotic eruption
with blistering of the skin.
On systemic examination, the patient was febrile and there was presence
of vesicobullous lesions over the face and neck region, sloughing of the
lips and oral mucosa, and within the oral cavity covering less than 10%
body surface area. [Figure 1a and 1b]. Nikolsky sign was positive.
The diagnosis of Steven Johnson Syndrome was made on clinical grounds by
a dermatological consultation.
His complete blood count suggested pancytopenia (hemoglobin – 10 g/dl,
total leukocyte count – 700/UL, platelet count – 8,000/UL). His blood
investigation suggested severe neutropenia (Absolute Neutrophil Count
=35). His serum albumin level was noted to be 2.8g/dl and his serum
alkaline phosphatase was 306U/L. His urine routine examination showed 3+
urine sugar. Other biochemical test results were within normal limits
initially.
The patient was admitted to the intensive care unit. Blood culture was
sent and the patient was started on supportive antibiotics with
injection Vancomycin and Gentamycin. In view of decreased counts,
injection filgrastim 50 μg was given subcutaneously once a day. The
other supportive cares included wound care, fluid and electrolyte
management, nutritional support, ocular care, temperature control and
pain management.
After stopping the chemotherapy, the patient’s rash started to improve
but he developed respiratory symptoms which further deteriorated and he
succumbed to his illness on the 6th day in spite of all best measures
being taken.