An 8-year-old girl for first-cousin parents, she is the second child among four girls of a Syrian family having a refugee-status at a camp in Sulaymaniyah, northern Iraq, since 2014. Our patient was born uneventfully in August 2010 and received BCG vaccine, according to the schedule at 7th day of age. Two months later, she developed ipsilateral axillary lymphadenitis followed by generalized lymphadenopathy. Meanwhile, features of disseminated BCG infection, including fever, weight loss, disseminated maculopapular rash, and hepatosplenomegaly, were manifested, and managed by a prolonged course of anti-TB medicines including isoniazid, and rifampin. According to the history taken from the mother, our patient had repeated episodes of non-specific illnesses, in form of relapsing/remitting maculopapular skin rash, oral thrush, respiratory infection, gastroenteritis, and urinary tract infections that were treated in an outpatient setting, in addition to one episode of meningitis treated at a hospital in Syria. At 4-year-old, as the family fled the war in Syria to a camp in northern Iraq, the child's condition was severely deteriorated and she became seriously ill with fever, night sweating, diarrhea, and poor appetite. Thus, she was referred to the intensive care unit at Hiwa Hospital in Sulaymaniyah, the northern province in Iraq. Upon admission she was toxic, cachexic, and feverish, with generalized lymphadenopathy including cervical, axillary, inguinal and epitrochlear lymph nodes. The lymph nodes were multiple, asymmetrical, and visibly enlarged with the biggest about (3.5 x 3 cm) at left axilla, firm in consistency, not tender, and discrete. The abdomen was distended with the presence of hepatosplenomegaly and ascites, in addition to right lung crepitation. The patients' growth parameters were below the third centile. Investigations showed an erythrocyte sedimentation rate (ESR) of 110 (normal range 3-13) millimeters/hour (mm/h), along with hypochromic microcytic anemia, leukocytosis, and high immunoglobulin-G assay. Ascetic fluid showed lymphocytic predominance with a serum-to-ascites albumin gradient of < 1.1 gm/dl, normal liver, and renal function tests. HIV and hepatitis screening were negative. Chest X-ray and computed tomography (CT) of the chest and abdomen showed a pulmonary consolidation at the right lower lung, in addition to mesenteric lymphadenitis disclosed by CT. Although microbiological and histopathological evaluations were not done, there was a high index of suspicion of mycobacterial infection, either in the form of relapsing disseminated BCG disease or active TB, based on the TB-prevalent situation at the area of the camp. Furthermore, the patient did not respond to an initial course of broad-spectrum antibiotics. Thus, she was treated empirically with 4 anti-TB medications for 12 months, including; isoniazid, rifampin, pyrazinamide, and streptomycin that was later changed to ethambutol. She showed a very good clinical and laboratory responses. Several months later, after stopping anti-TB therapy, she relapsed with generalized lymphadenopathy and maculopapular skin rash (Figure 1).
She also had episodes of abdominal pain and bloody diarrhea, disturbed sleep, and weight loss. Our patient underwent several excisional biopsies from axillary, cervical, and groin lymph nodes, in Syria and in Iraq, but the results were non-conclusive. Moreover, during the periods of suspected infection with leukocytosis and lymph node neutrophilic infiltration, culture was not regularly done, mostly because of the limited laboratory facilities and being treated in an out-patient setting. There was no history of BCG disease or TB, among family members. On most occasions the patient had an ESR of >= 100 mm/h, hypochromic microcytic anemia, leukocytosis, neutrophilia, lymphopenia with hypercellular marrow examination, and low CD3 and CD4 by flowcytometry. Antinuclear antibody, in addition to toxoplasmosis, rubella, cytomegalovirus, herpes simplex, HIV, and syphilis, as well as the culture for TB, were all negative. Thyroid, liver, and renal function tests were normal.