20/11/2021
1st Admission at AIMS, India (kochi):
(16/12/2020- 02/01/2021)
She was admitted to the NICU of AIMS .Her investigation showed bicytopenia (anemia with leukopenia)😢, with agranulocytosis ,mildly elevated CRP, hyperferritinemia and hypertriglyceridemia. Hence she was suspected to have a hemophagocytic process and in the presence of panniculitis like lesions, a possibility of cytophagic histiocytic panniculitis was considered.
Born marrow aspiration and biopsy and deep skin biopsy was performed under general anesthesia on 17/12/2020.
Meanwhile in view of agranulocytosis, she was started empirically on parenteral antibiotics.
Born marrow aspiration showed dilute marrow(no opinion possible)
Bone marrow biopsy showed 4-5 normocellular marrow spaces showing myeloid maturation arrest and increased megakaryocytes.
Skin biopsy showed atypical mononuclear infiltrate with S 100, dot like EMA positivity and high ki index favoring a histiocytic lineage. A probable diagnosis of cutaneous interdigitating dendritic cell sarcoma was made after correlating with the IHC findings.
It was decided to observe her for a period of 3 months with an advice to follow up every 10 days with complete blood count.
Her counts improved (Hb – 8.3 gm%, TC 10230, ANC 1640, Plt 5.48 L per cu mm). Hence she was discharged on 02/01/2021. Her weight at the time of discharge was 3.1 kg.
However, she remained in follow up due to possibility of relapse of the lesions. During the follow up she has been showing possible ongoing recurring neutropenia. As her neutrophil counts were fluctuating, doctors suspected cyclical neutropenia / congenital neutropenia.
- Her Immunoglobin levels were normal. However, T, B. NK cell enumeration in newborn period showed low absolute number of B cells.
- NGS repoted as no clinically relevant mutation detected.
While she was in India she had rashes on her both cheeks and redness on the back of her head lead to alopecia(note: Alopecia resolved on its own and rashes on the face faded away by applying Seba Med cream).
After that, as she was stable, she went back to Maldives on 25/03/2021.
