The association of systemic lupus erythematosus SLE and acute lymphoblastic leukemia ALL is rarely reported in literature for the pediatric age group. Usually, SLE precedes the onset of lymphoproliferative disease, but the malignancy can occur earlier or even simultaneously.
We report a case of 15 year old male child 12 year back diagnosed as SLE, recently on routine investigation incidentally diagnosed as ALL. SLE is an autoimmune disease characterized by inflammation of blood vessels and connective tissue resulting in multisystem involvement. The clinical manifestations are extremely variable. Childhood SLE affects girls more often than boys , even in the prepubescent age group The childhood SLE is usually more severe and has a poorer prognosis than adult.
The association of SLE and acute lymphoblastic leukemia ALL in pediatric patients only few cases reported in literature. Usually, SLE precedes the onset of lymphoproliferative disease, but the neoplasia can occur earlier or even simultaneously. A 15 year old male child was a known case of lupus nephritis, on routine follow up complete blood count reveled high TLC.
Peripheral blood film analysis suggests acute lymphoblastic leukemia Figure 1. The flocytometry suggests B cell acute lymphoblastic leukemia.
The karyotype of child was 46XY and no structural abnormality, no hypo or hyper diploidy was seen Figure 2. The Philadelphia chromosome was not evident in any of the metaphases. There was no hypocomplementemia. Blood culture and sensitivity showed no growth. Skin biopsy showed vascular and perivascular infiltration of polymorphonuclear leukocytes with formation of nuclear dust, extravasation of erythrocytes, and fibrinoid necrosis of the vessel walls; a picture consistent with leukocytoclastic vasculitis.
Intravenous methyl-prednisolone 3-gram over three days was started. The patient was discharged with marked improvement of fever, skin lesions Figure 3 , general condition and all the initially abnormal laboratory investigations. He was scheduled for a follow-up visit after one month. Figure 3: Patient's pictures on discharge showing marked improvement of all skin lesions. View Figure 3. One month later the patient was readmitted to the hospital with high grade fever and extensive skin crusty bullous lesions over knees and legs Figure 4.
Investigations revealed pancytopenia with relative lymphocytosis and some atypical lymphocytes. The patient was transferred to The National Cancer Institute and missed his follow up.
Figure 4: Multiple bulbous bullous-like lesions with crusty areas over knee and livedo like lesions over legs. View Figure 4. Paraneoplastic syndromes are the manifestations of an underlying malignancy that may be hidden.
The paraneoplastic rheumatic disorders include a wide spectrum of manifestations for example; arthritis, myositis, lupus-like syndrome and vasculitis. They may be an initial presentation, in such cases the diagnosis may be difficult and needs a high index of suspicion. The conclusions in the systemic review and meta-analysis by Song and colleagues are impactful to the care of patients with SLE. These findings afford rheumatology providers with evidence that can be used to inform patients regarding their cancer risks.
Additionally, it emphasizes the importance of ensuring that SLE patients are receiving the necessary cancer screening as indicated by organizations such as the American Cancer Society and the U. Preventive Services Task Force.
Healio News Rheumatology Lupus. Source: Song L, et al. Read next. January 02, Receive an email when new articles are posted on. All information contained within the Johns Hopkins Lupus Center website is intended for educational purposes only.
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