[CIS PIDD] [cis-pidd] Splenomegaly and B cell expansion

[Harville, Terry O]

Richard,

I have seen this pattern with congenital CMV.

Terry Harville MD PhD
-Medical Director, Special Immunology Laboratory
-Medical Director, Histocompatibility Laboratory
-Medical Director, Immunogenetics and Transplantation Laboratory
-Specialist in Pediatric Immunology and Rheumatology
Departments of Pathology and Laboratory Services and Pediatrics
University of Arkansas for Medical Sciences
4301 West Markham
Mail Slot #502
Little Rock, AR  72205-7199

Work Phone 1.......................................501.686.7257
Work Phone 2.......................................501.526.7511
Work Phone 3.......................................501.686.7556
Work Fax 1.........................................501.686.7443
Work Fax 2.........................................501.526.4621

Email...................................harvilleterryo at uams.edu

Special Immunology Laboratory......................501.364.1804
Histocompatibility Laboratory......................501.686.7257
Immunogenetics and Transplantation Laboratory......501.686.7374

From: Richard Wasserman [mailto:drrichwasserman at gmail.com]
Sent: Saturday, November 23, 2013 3:25 PM
To: CIS-PIDD
Subject: [cis-pidd] Splenomegaly and B cell expansion

We are seeing a now 20 day old male with low/very low TRECs on SCID screen who has been well but had splenomegaly (homogeneous on ultrasound) without hepatomegaly or adenopathy. Mother's great maternal uncle died at 6 weeks of age.

Initial CBC -
WBC 44.3
HGB 11.4
Platelets 18K

Segs 36%
Bands 2%
Lymphs 37%
Atypcial lymphs "a few"
Monos 14%
Eos 1%

We initially thought he had leukemia - bone marrow showed "exuberant hematogone hyperplasia" with aberrancy.

Lymphocyte markers:
CD3 8.4% abs 1377
CD4 6.4% abs 1051
CD8 1.6% abs 257
CD 16/56 19.6% abs 3214
CD19 70.7% abs 11592

Extended flow analysis
2.1% variably sized immature B-cells with the following phenotype: CD34+, CD13-, CD33-, CD19 dim+, CD20-, CD22 dim+to-, CD38 uniformly +, CD45 moderately +, CD79a+, CD117-, TdT+, MPO-, other myeloid and lymphoid antigens predominantly -.
48% left shifted granulocytic elements
21% monocytes
4.9% CD34+ myeloblasts without overt immunophenotypic aberation
2.1% small, mature T lymphocytes CD4:CD8=4.5:1
2.6% NK cells
17% small, mature polytypic B lymphocytes (kappa:lambda 0.88:1)
COMMENT - a population of immature B-cells with immunophenotypic features of stage I hematogones that show no aberrant expression of myeloid antigens. Expansion of unremarkable myeloblasts in a background of left-shifted granulocytes. These findings are likely reactive.

IgM=42mg/dL

Pending lab: cytogenetics, FISH, CMV PCR, repeat TREC, PHA stimulation

Could this be SCID with maternal B-cells? Why the thrombocytopenia? Why the left-shifted granulocytosis?

Richard Wasserman
--
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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