Ann Lab Med.  2013 May;33(3):208-211. 10.3343/alm.2013.33.3.208.

A Case of IgM Deficiency with B Cell Deficiency Detected by ABO Discrepancy in a Patient with Acute Osteomyelitis

• Jung CL
• Cha MK
• Jun BH
• Hong KS

• KMID: 1781329
• DOI: http://doi.org/10.3343/alm.2013.33.3.208

Abstract

ABO discrepancy refers to an inconsistency between red cell and serum typings and has various causes, including hypogammaglobulinemia. IgM deficiency is a rare disorder that may accompany several conditions such as infection and autoimmune disorders. Here, we describe a case of IgM deficiency discovered during the evaluation of an ABO discrepancy in a 16-yr-old Korean boy. ABO blood grouping showed that while his cell type was O+, serum typing detected only anti-A (3+). Anti-B was not detectable at room temperature but was graded at 1+ at 4degrees C. ABO genotyping revealed an O/O genotype. His serum IgG, IgA, and IgM concentrations were 770 mg/dL (reference range: 800-1,700 mg/dL), 244 mg/dL (reference range: 100-490 mg/dL), and 13.5 mg/dL (reference range: 50-320 mg/dL), respectively. He was diagnosed with acute osteomyelitis on the basis of clinical presentation and imaging studies. The symptoms gradually improved within 3 weeks of treatment. However, the ABO discrepancy and IgM deficiency persisted even 6 months after recovery and lymphocyte subset analysis revealed CD19+ B cell deficiency. To the best of our knowledge, IgM deficiency detected by ABO discrepancy in a patient with acute osteomyelitis has not been reported before.

Keyword

IgM deficiency; B cell deficiency; ABO discrepancy; Acute osteomyelitis

MeSH Terms

ABO Blood-Group System/genetics
Acute Disease
Adolescent
B-Lymphocytes/cytology/immunology/metabolism
Bone and Bones/radionuclide imaging
Genotype
Humans
Immunoglobulin A/blood
Immunoglobulin G/blood
Immunoglobulin M/blood
Immunologic Deficiency Syndromes/complications/*diagnosis
Knee/radionuclide imaging
Magnetic Resonance Imaging
Male
Osteomyelitis/complications/*diagnosis
Radiopharmaceuticals/diagnostic use
ABO Blood-Group System
Immunoglobulin A
Immunoglobulin G
Immunoglobulin M
Radiopharmaceuticals

Figure

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  Fig. 1 T2-weighted magnetic resonance image of the right knee, sagittal section. The image demonstrated bone marrow signal deterioration at the distal femoral metaphysis (short arrow) and anterior crucial ligament tibial attachment (long arrow), suggesting chronic recurrent multifocal osteomyelitis or acute osteomyelitis.

• 

  Fig. 2 Bone scan revealed increased uptake only in the metaphysis of the right femur (arrows).Abbreviations: ANT, anterior; POS, posterior; RT, right; LT, left.

Reference

1. Goldstein MF, Goldstein AL, Dunsky EH, Dvorin DJ, Belecanech GA, Shamir K. Selective IgM immunodeficiency: retrospective analysis of 36 adult patients with review of the literature. Ann Allergy Asthma Immunol. 2006; 97:717–730. PMID: 17201230.

2. Yel L, Ramanuja S, Gupta S. Clinical and immunological features in IgM deficiency. Int Arch Allergy Immunol. 2009; 150:291–298. PMID: 19494527.

3. Goldstein MF, Goldstein AL, Dunsky EH, Dvorin DJ, Belecanech GA, Shamir K. Pediatric selective IgM immunodeficiency. Clin Dev Immunol. 2008; 2008:624850. PMID: 19043622.

4. Suh IB, Chang EA, Kim HJ, Kang CD, Lee SJ, Kim TS, et al. Two cases of ABO discrepancy due to hypogammaglobulinemia. Korean J Blood Transfus. 2003; 14:240–245.

5. Oh SH, Kang CI, Kim J, Park TS. ABO discrepancy in a young Korean serviceman with common variable immunodeficiency. Ann Hematol. 2010; 89:629–630. PMID: 19844713.

6. Lee JH, Jung KH, Han KS, Han BY, Yoon JH, Chun SA. A case of group O without anti-A,B due to hypogammaglobuluinemia. Korean J Blood Transfus. 1996; 7:269–273.

7. Park TS, Oh SH, Choi JC, Kim HH, Park JH, Lee EY, et al. A case of agammaglobulinemia detected by ABO discrepancy in a 13-yr-old girl. Korean J Lab Med. 2002; 22:364–366.

8. Ha JS, Kim EJ, Chun HJ, Kim JR, Jeon DS. Three cases of multiple myeloma showing ABO discrepancy. Korean J Blood Transfus. 1998; 9:289–293.

9. Hyun J, Chang HE, Heo SR, Song SH, Park KU, Song J, et al. ABO genotyping using a multiplex single-base primer extension reaction. Korean J Blood Transfus. 2007; 18:79–88.

10. Gathmann B, Binder N, Ehl S, Kindle G. ESID Registry Working Party. The European internet-based patient and research database for primary immunodeficiencies: update 2011. Clin Exp Immunol. 2012; 167:479–491. PMID: 22288591.

11. Moise A, Nedelcu FD, Toader MA, Sora SM, Tica A, Ferastraoaru DE, et al. Primary immunodeficiencies of the B lymphocyte. J Med Life. 2010; 3:60–63. PMID: 20302197.

12. Al-Herz W, Bousfiha A, Casanova JL, Chapel H, Conley ME, Cunningham-Rundles C, et al. Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency. Front Immunol. 2011; 2:54. PMID: 22566844.

13. Notarangelo LD, Fischer A, Geha RS, Casanova JL, Chapel H, Conley ME, et al. Primary immunodeficiencies: 2009 update. J Allergy Clin Immunol. 2009; 124:1161–1178. PMID: 20004777.

14. Zhang Q, Davis JC, Dove CG, Su HC. Genetic, clinical, and laboratory markers for DOCK8 immunodeficiency syndrome. Dis Markers. 2010; 29:131–139. PMID: 21178272.

15. Wipff J, Adamsbaum C, Kahan A, Job-Deslandre C. Chronic recurrent multifocal osteomyelitis. Joint Bone Spine. 2011; 78:555–560. PMID: 21441060.

16. Makay B, Unsal E, Anal O, Güne D, Men S, Cakmakçi H, et al. Chronic recurrent multifocal osteomyelitis in a patient with selective immunoglobulin M deficiency. Rheumatol Int. 2009; 29:811–815. PMID: 19034453.

17. Offiah AC. Acute osteomyelitis, septic arthritis and discitis: differences between neonates and older children. Eur J Radiol. 2006; 60:221–232. PMID: 16971078.

18. Gupta S, Agrawal S, Gollapudi S. Selective IgM deficiency with T cell defects and Mycobacterium Avium Complex (MAC) infection. Open Immunol J. 2012; 5:8–12.

19. Mazda T, Yabe R, NaThalang O, Thammavong T, Tadokoro K. Differences in ABO antibody levels among blood donors: a comparison between past and present Japanese, Laotian, and Thai populations. Immunohematology. 2007; 23:38–41. PMID: 17425414.

20. Cho CH, Kim HN, Yun SG, Choi GR, Choi JY, Kim JS, et al. Evaluation of ABO antibody titration using tube and column agglutination techniques. Lab Med Online. 2011; 1:57–63.