Transfusion associated graft versus host disease

Editor's Notes

1. On admission mild anaemia (? Secondary to peptic ulcer disease) with mild lymphocytopenia NL Lymphocyte count – 1000-3000/uL
2. Bilirubin – Total - 0.2-1.2 mg/dL direct bilirubin is 0.1-0.4 mg/dL ALP – 44-147IU/L Amylase 23-85U/L & Lipase 0-160U/L(acute pancreatitis, gallstones,peptic ulcer, CA)
3. Cultures – Bacterial and fungal AMA – primary billiary cirrhosis
4. Haemophagocytic syndrome - dysfunction of cytotoxic T cells and NK cells. This T cell/NK cell dysregulation causes an aberrant cytokine release, resulting in proliferation/activation of histiocytes with subsequent haemophagocytosis. Histiocytic infiltration of the reticuloendothelial system results in hepatomegaly, splenomegaly, lymphadenopathy and pancytopenia ultimately leading to multiple organ dysfunctions.
5. Microsatellites - short tandem repeats (STRs) - repetitive DNA in which certain DNA motifs (ranging in length from 2–5 base pairs) are repeated, typically 5–50 times.[
6. Skin – like in post admission day 4 report
7. Most of our understanding of the pathophysiology of GVHD comes from the hematopoietic stem cell transplant (HSCT) setting.
8. Typically, in immunocompetent hosts,viable T lymphocytes are destroyed by the recipient’s immune system. In susceptible patients, whether immunocompetent or with congenital or acquired cellular immune deficiency,transfused T cells are not destroyed. Ex – inherited -severe combined immuno deficiency, acquired-Hodgkins lymphoma
9. A cytokine storm, also known as cytokine cascade and hypercytokinemia, is a potentially fatal immune reaction consisting of a positive feedback loop between cytokines and white blood cells, The additional presence of “danger signals”—such as lipopolysaccharide (LPS) or infl ammatory cytokines—help APCs activate T cells and the presence/absence of “danger signals” may make the difference between an immune response and tolerance.
10. The additional presence of “danger signals”—such as lipopolysaccharide (LPS) or inflammatory cytokines—help APCs (dendritic cells) activate T cells and the presence/absence of “danger signals” may make the difference between an immune response and tolerance.10
11. Mitogens (chemicals commence cell devision) are very potent stimulators of T-cell activation and proliferation independent of their antigenic specificity.(3) It has been suggested that mitogens can induce T-cell proliferative responses even if they are incapable of responding adequately to antigenic (physiologic) stimuli.
12. Typical course of the disease
13. Vacuolar interface dermatitis - dermatitis where degenerative changes occur at the dermoepidermal junction, with lymphophatic inflammation at the epidermis and dermis.
14. STR/microsatellite - tract of repetitive DNA in which certain DNA motifs (ranging in length from 2–5 base pairs) are repeated, typically 5–50 times Tandem repeats - pattern of one or more nucleotides is repeated and the repetitions are directly adjacent to each other. VNTR/minisatellite
15. Anti-thymocyte globulin (ATG) is an infusion of horse or rabbit-derived antibodies against human T cells
16. Using properly irradiated blood components would prevent this disease; however, without completely knowing which patients are at risk, all transfused cellular blood products would have to be irradiated for adequate prevention. The cost and logistics of such a task and the radiation effects of shortening the shelf life of the blood supply and increasing plasma potassium in RBC units make the value of widespread irradiation questionable given the uncertain benefits for such an apparently rare disease
17. The transfused lymphocytes recognized the patient as foreign; however, the patient recognized the transfused lymphocytes as self. Only a few other reported cases of TA-GVHD demonstrate only a class II HLA mismatch. If a patient develops a skin rash, diarrhea, liver failure, and bone marrow aplasia shortly after blood transfusion,TA-GVHD should be considered in the differential diagnosis.