Abstract
Answers 1. The changes in granulocyte nuclei morphology along with the presence of immature forms in peripheral blood (after the exclusion of myelodysplastic syndrome, leukemia, and infection) allowed us to diagnose the pseudo Pelger–Huet anomaly (PPHA). 2. A unilobed neutrophil in the patient’s peripheral blood. 3. It was decided to administer another dose of rituximab (Rtx), which resulted in the maintenance of CD20 lymphocyte depletion with subsequent remission of the nephrotic syndrome. This also allowed us to terminate corticosteroid therapy. Discussion
Highlights
CLINICAL QUIZOccurrence of neutrophil dysplasia in the course of severe nephrotic syndrome in a 12-year-old boy on immunosuppressive therapy: Answers
Pelger–Huët anomaly (PHA) is a very rare, multi-factorial disorder of granulocytopoiesis that manifests itself in reduced nuclear segmentation and excessive chromatin condensation in neutrophils [1,2,3,4]
There are many hypotheses that suggest a possible mechanism of an impaired process of granulocyte nuclei segmentation in pseudo Pelger–Huët anomaly (PPHA), e.g., individual variation of inosine-5′-monophosphate dehydrogenase (IMPDH) activity, which has an influence on adverse effects of MMF [6]
Summary
Occurrence of neutrophil dysplasia in the course of severe nephrotic syndrome in a 12-year-old boy on immunosuppressive therapy: Answers. 1. The changes in granulocyte nuclei morphology along with the presence of immature forms in peripheral blood (after the exclusion of myelodysplastic syndrome, leukemia, and infection) allowed us to diagnose the pseudo Pelger–Huët anomaly (PPHA). 3. It was decided to administer another dose of rituximab (Rtx), which resulted in the maintenance of CD20 lymphocyte depletion with subsequent remission of the nephrotic syndrome. It was decided to administer another dose of rituximab (Rtx), which resulted in the maintenance of CD20 lymphocyte depletion with subsequent remission of the nephrotic syndrome This allowed us to terminate corticosteroid therapy.
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