The prevalence of initial and subsequent RBC alloimmunization in Period 2 was lower than that in Period 1; however, overall prevalence remained high. We recommend leukoreduced, hemoglobin S-negative Rh and Kell PAM RBCs for transfusion of patients with SCD. Component and recipient factors affecting alloimmunization should be studied further.
Dural extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a rare entity without an associated recurrent genetic abnormality. Only one case has been described in a woman with history of breast carcinoma without a known genetic predisposition. Here, we report a case of a 56-year-old woman heterozygous for XRCC2 mutation with a history of Graves’ disease and bilateral breast carcinomas, who was found to have a diffusely infiltrative extra-axial mass in the high parietal convexity with infiltration into the adjacent superior sagittal sinus. The morphologic, immunophenotypic, and molecular findings were diagnostic of MALT lymphoma. Staging bone marrow demonstrated involvement by the neoplasm. Although the study was limited to only the clinically significant laboratory evaluation, it may serve as an important addition to the current knowledge of the pathogenic potential of a loss of function mutation in this rarely reported cancer predisposition gene.
Chimerism testing by short tandem repeats (STRs) is used to monitor engraftment after allogeneic hematopoietic stem cell transplantation (HSCT). Generally, STR alleles are stable and transferred from parent to child or from donor to recipient. However, 3 cases did not follow this norm. Additional work-up with help from forensic literature solved these mysteries.
In case 1, the patient received HSCT from his son. The son shared STR alleles in 22/23 loci except Penta E, which was explained by repeat expansion in the son.
In case 2, the patient had been in remission for 14 years after HSCT for lymphoma and developed repeat expansion in CSF1PO in granulocytes.
In case 3, a pre-HSCT patient demonstrated 3 alleles, with 2 peaks taller than the third, in the FGA locus (chromosome 4). A combination of a triallelic variant and leukemia-associated trisomy 4 explained the finding. STR number variants are rare and clinically inconsequential but can overlap malignancy-associated, clinically significant changes.
Introduction/Objective
Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a curative treatment option for patients with myelodysplastic syndrome (MDS). Nonetheless, a large proportion of patients with MDS experience disease relapse. Declining donor chimerism and detection of recurrent gene mutations have been used as indicators of graft failure and recurrent disease. Blood Bank serologic findings have rarely been described as first indicators of disease relapse in this setting and could potentialy add to engraftment and relapse surveillance testing.
Methods/Case Report
A 72-year-old, ABO group O, RhD positive male with history of anti-Fyb alloimmunization underwent allo-HSCT from an ABO group B, RhD negative, Fyb positive donor as part of the treatment for MDS. Successful engraftment was achieved, and the patient’s red blood cell phenotype transitioned to ABO group B, RhD negative, Fyb positive. Two years following allo-HSCT, the patient received chemotherapy for recurrent cholangiocarcinoma. Supportive blood component transfusions were provided, all of which typed as RhD negative. However, new antibody with anti-D specificity was detected in serum while the patient still typed as ABO group B, RhD negative, preceding anti-D and later recurrent anti-Fyb detection in eluate, and prompting further chimerism testing. Declining donor chimerism was noted (72% donor, compared to >98% donor on prior chimerism testing). Chemotherapy and donor-lymphocyte infusion were initiated.
Results (if a Case Study enter NA)
NA
Conclusion
The early detection of de novowlvw anti-D was most consistent with resurgence of patient’s erythroids within the bone marrow in the presence of donor’s immune system. This was followed by sufficient peripheralization of patient’s red blood cells and detection of anti-D in the eluate. Lastly, the switch to recipient’s immune system is evidenced by recurrent detection of anti-Fyb in the eluate. This case, therefore, emphasizes the utility of Blood Bank serology in raising suspicion for disease relapse and guiding further allo-HSCT patient management. More systematic use of Blood Bank serology may serve as a time- and cost-effective adjunct to the current strategies employed for detection of disease recurrence in allo-HSCT recipients.
Complement component 4d (C4d) is a degradation product of the classical pathway. C4d immunohistochemistry is used for the diagnosis of complement-dependent antibody-mediated rejection (ABMR) in posttransplant renal biopsies. We studied serum C4d levels, measured by a noninvasive and quantitative test, in two posttransplant patients (A and B) with worsening renal function and biopsies demonstrating peritubular capillaritis and glomerulitis. Serum samples from patients A and B were obtained within 24 hours of biopsy results indicative of ABMR. The patients' sera and eight controls were stored at -20°C before testing the samples. A commercially available enzyme-linked immunosorbent assay kit was used to measure serum concentrations of C4d (Uscn Life Science Inc, Hubei, China), following the manufacturer's instructions. C4d immunohistochemical staining was performed on formalin-fixed, paraffin-embedded renal biopsy specimens. In addition, the patients' sera were tested for anti-HLA Class I and Class II donor-specific antibodies (DSA) by solid-phase immunoassay (Luminex, Austin, TX). Patient A, with diffuse peritubular capillary C4d reactivity of greater than 75%, had a serum C4d level of 4.4 mg/L. Patient B, with 0% peritubular capillary C4d reactivity, had a serum C4d level of 1.7 mg/L. Both of the patients' serum C4d results were compared to the control group (mean ± SD), 2.4 ± 0.66 mg/L, range 1.6-3.3 mg/L. Patient A had two strong anti-class I DSA (both to the B-loci), and two weak anti-class II DSA (DQB1 and DQA1). Patient B had one moderate anti-Cw DSA. Serum C4d levels of patients A and B were consistent with biopsy C4d staining and presence of DSA. The measurement of serum C4d levels may serve as a quantitative test of complement-dependent ABMR in renal transplant recipients.
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