Hemophagocytic lymphohistiocytosis in a newborn infant born to a mother with Sjögren syndrome antibodies

Abstract

We encountered a neonatal patient with hemophagocytic lymphohistiocytosis (HLH) whose mother was positive for anti-Ro/SSA and anti-La/SSB antibodies. Complete atrioventricular block was found in a male patient at 29 weeks of gestation. The patient was born at 40 weeks of gestation. He showed severe circulatory disturbance at 22 h after the birth, and he also had elevated serum levels of aspartate aminotransferase (1027 IU l(-1)), alanine aminotransferase (121 IU l(-1)), lactic dehydrogenase (3490 IU l(-1)), ferritin (9769.7 ng ml(-1)) and soluble interleukin-2 (IL-2) receptor (3230 U ml(-1)). We could not find any known HLH genetic abnormality in the patient, but he fulfilled seven of the eight criteria for HLH. Serum levels of IL-6 and IL-8 had been already elevated in his cord blood, and serum levels of granulocyte-macrophage colony-stimulating factor and IL-8 were significantly increased on the second day of life. His symptoms regressed with the administration of hydrocortisone. We presumed that transplacental transfer of maternal antibodies could be related to the occurrence of HLH.

Neonatal Haemophagocytic Lymphohistiocytosis Associated with Maternal Adult-Onset Still's Disease

“…Although the exact mechanism in our patient cannot be fully elucidated, it is plausible that immunological factors such as auto-antibodies, antigens, or inflammatory mediators transferred vertically from the mother may have triggered similar immunological responses in the infant. The temporal relationship of disease onset and the self-limiting nature of the inflammation suggest that such transient factors induced an exaggerated pro-inflammatory reaction similar to the case described in maternal Sjogren syndrome [4]. The latter case also had an intense inflammatory response which was controlled by systemic hydrocortisone and without the need for transplantation.…”

“…Secondary HLH is usually associated with infection [2,3], malignancy, and autoimmune or rheumatological conditions [4]. Although the exact mechanism in our patient cannot be fully elucidated, it is plausible that immunological factors such as auto-antibodies, antigens, or inflammatory mediators transferred vertically from the mother may have triggered similar immunological responses in the infant.…”

“…It has been conventionally classified into primary familial HLH and secondary acquired disease [1,2,3,4]. Oftentimes, neonatal presentation is due to familial disease [1] or pathogen-associated secondary cases [2,3].…”

Kawasaki disease in a preterm neonate: Case report and cytokine profile

“…In the present case, KD developing in a preterm neonate soon after birth was treated with high‐dose IVIG, which was effective in blocking the production of all the cytokines at once. The concentration of 17 types of cytokines in a small volume of serum was measured using a highly sensitive fluorescence microsphere system (Bio‐Plex suspension array system; Hercules, CA, USA) before and after IVIG treatment (Table ) . The control group consisted of eight neonates with slightly low birthweight ( n = 5), a diabetic mother ( n = 2), or with hyperbilirubinemia caused by breast‐feeding ( n = 1) .…”

Cytokine profile in two siblings with neonatal lupus erythematosus

“…We previously reported the clinical course of the first male infant (patient 1) in detail . Briefly, his general condition at birth was stable in spite of CAVB at 70 beats/min.…”