[CIS PIDD] [cis-pidd] A child with neutropenia

[CIS-PIDD]

Dear all

We hope to get opinions and suggestions for a 2-year-old girl with
persistent fever and neutropenia who was admitted to our ward in the recent
past.

She presented with persistent high-grade fever without focus for a
1.5-month duration. She was evaluated for pyrexia of unknown origin before
she landed to us. Clinically apart from fever, she had a vaginal mucosal
ulcer (1x1 cm), and a small ecthyma-like lesion in the bone marrow
aspiration site. No hepatosplenomegaly.

Her blood counts revealed consistent neutropenia for last one month.
*Absolute neutrophil counts in range 50-200. Elevation in monocyte
percentages is also noted in the differential counts (20-30%).* No
eosinophilia or basophilia. Total white cell count (5000- 6500/cu.mm),
hemoglobin, and RBC counts are normal. Platelet counts are elevated
(5.4-6.3 lakhs/cu.mm). * The peripheral smear also showed severe
neutropenia. *

*I should make a note here that the counts done a year back showed no
neutropenia (White cells- 8,600; Differential counts- 32% polymorphs, 60%
lymphocytes, 4% monocytes, 4% eosinophils-4%; platelets- 3.04 lakhs).*

Bone marrow aspiration and biopsy done (repeated twice) for the evaluation
of neutropenia showed *a hypocellular marrow (overall cellularity: 60-70%)
with early maturational arrest in granulocytic series* (blasts-06%,
promyelocytes-01%, myelocytes-03%, mature polymorphs- 01%)

Malignancy, tuberculosis- ruled out. Viral serologies- CMV, EBV, HIV, Hep
B, Hep C are negative. Immunoglobulin profile- normal. CD 3, CD19, CD56,
CD4, CD8 by flow cytometry are normal. Blood cultures were consistently
sterile. Bone marrow bacterial and fungal cultures were sterile. Serum
procalcitonin was normal. Chest radiograph and high resolution CT chest
showed no pneumonia. Ultrasound abdomen is normal. No deep space neck or
perianal abscess.

She received IV vancomycin, meropenem for 14 days, IV linezolid,
piperacillin tazobactum, and amikacin for next 14 days. She also received
empirical IV amphotericin for 14 days. However, the fever was persistent
despite multiple antimicrobials and amphotericin.

She was initiated on subcut G-CSF at day 15 of her illness. Rise in ANC was
not noted at the doses of 5, 10, 15, or 20 mics. Finally her ANC started to
rise briskly at 25 microgram per kg/day of G-CSF (ANC went upto 12,000!!!).
As the ANC started to rise, her fever subsided completely. Currently she is
doing fine on 20 micrograms daily of G-CSF.

Specific queries for this patient:

1. Does the clinical presentation sound like a congenital neutropenia? The
presence of early maturational arrest in the marrow, persistent
neutropenia, and requirement of large doses of G-CSF are the points we
considered favoring for congenital neutropenia. However, the ANC done a
year back was normal for the patient, which we felt is little unusual for a
severe congenital neutropenia.

2. Does this patient require evaluation for autoimmune neutropenia
(measurement of anti-neutrophil antibodies), given the severe maturational
arrest in the bone marrow?

3. The fever that persisted despite multiple antimicrobials and antifungals
for a month subsided dramatically with rise in neutrophil counts after
G-CSF therapy. Has anyone seen this kind of response to fever in the
neutropenic patients?

Thank you.

Vignesh P
MD Pediatrics,
DM resident in Pediatric Clinical Immunology and Rheumatology (Jan 2015-
Dec 2017),
Allergy Immunology Unit, Advanced Pediatrics Center,
Postgraduate Institute of Medical Education and Research,
Chandigarh, India. 160012.
E mail: vigimmc at gmail.com
Phone no: +91-9592047009, +91-9944547009

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