Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc
Journal website https://www.journalmc.org

Case Report

Volume 14, Number 9-10, October 2023, pages 327-331


A Double Hit to Ubiquitination Leading to a New Diagnosis of VEXAS Syndrome

Figure

Figure 1.
Figure 1. (a) Bone marrow biopsy showing numerous precursor cells including metamyelocytes and promyelocytes with intracellular vacuoles (white arrow). (b) Bone marrow biopsy showing hypercellular bone marrow (black arrow).

Tables

Table 1. Initial Laboratory Values
 
LabValueReference range
WBC: white blood cell; Hb: hemoglobin; MCV: mean corpuscular volume; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein.
WBC14.3 g/dL4.5 - 11 × 103 g/dL
Hb10.1 g/dL12 - 17.5 g/dL
MCV100.8 fL80.0 - 100.0 fL
Platelet232 × 103/µL140 - 450 × 103/µL
Lymphocytes12.2% lymphocytes25.0-43.0%
Metamyelocytes3.5%0%
Myelocytes3.5%0%
Promyelocytes0.9%0%
Iron4045 - 180 µg/dL
Ferritin1,042.922 - 335 ng/mL
Transferrin< 70215 - 365 mg/dL
ESR900 - 20 mm/h
CRP490 - 10 mg/L

 

Table 2. Autoimmune Workup
 
LabValueReference range
TPO: thyroperoxidase; RNP: ribonucleoprotein; Hb: hemoglobin; G6PD: glucose-6-phosphate dehydrogenase; PNH: paroxysmal nocturnal hemoglobinuria.
TPO114< 9 IU/mL
RNP30.0 - 0.9 AI
G6PD23.87.0 - 20.5 U/g Hb
PNH assayNo flow cytometric evidenceNegative