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

[Richard Wasserman]

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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