TY - JOUR
T1 - Lack of muco-cutaneous signs of toxic shock syndrome when t cells are absent
T2 - S. aureus shock in immunodeficient adults with multiple myeloma
AU - Kamel, N. S.
AU - Banks, M. C.
AU - Dosik, A.
AU - Ursea, D.
AU - Yarilina, A. A.
AU - Posnett, D. N.
PY - 2002
Y1 - 2002
N2 - Staphylococcal toxic shock syndrome (TSS) is an acute life threatening disease. The diagnosis can be made clinically based on diagnostic criteria. The clinical manifestations are caused in large part by the release of high levels of T-cell-derived cytokines as a result of potent toxins, also called superantigens (SAg), produced by Staphylococcus aureus, but it is not clear which clinical symptoms/signs are strictly T-cell dependent. Here, we report on three adults with multiple myeloma (MM) presenting with S. aureus sepsis/shock, and two patients with typical TSS. The MM patients had compromised humoral immunity because of depression of normal immunoglobulin (Ig) levels at the expense of the M protein. In addition, their T cells were absent due to high dose chemotherapy initiated for bone marrow transplantation. The MM cases lacked mucosal hyperemia, erythroderma and desquamation, but were otherwise indistinguishable from the TSS cases. All patients grew S. aureus and in each case, SAg genes were detected by PCR. In several cases, the plasma contained biological SAg activity resulting in Vβ specific proliferation of indicator T cellsin vitro. The same specific activity was observed with the supernatant fluids of S. aureus broth cultures from the respective bacterial isolates. This confirms the presence of bio-active toxins in the plasma but did not lead to full blown TSS when T cells were lacking. Thus, S. aureus sepsis/shock can be clinically distinguished from typical TSS, and we suggest that muco-cutaneous manifestations of TSS are the most telling signs of massive T-cell-dependent cytokine release.
AB - Staphylococcal toxic shock syndrome (TSS) is an acute life threatening disease. The diagnosis can be made clinically based on diagnostic criteria. The clinical manifestations are caused in large part by the release of high levels of T-cell-derived cytokines as a result of potent toxins, also called superantigens (SAg), produced by Staphylococcus aureus, but it is not clear which clinical symptoms/signs are strictly T-cell dependent. Here, we report on three adults with multiple myeloma (MM) presenting with S. aureus sepsis/shock, and two patients with typical TSS. The MM patients had compromised humoral immunity because of depression of normal immunoglobulin (Ig) levels at the expense of the M protein. In addition, their T cells were absent due to high dose chemotherapy initiated for bone marrow transplantation. The MM cases lacked mucosal hyperemia, erythroderma and desquamation, but were otherwise indistinguishable from the TSS cases. All patients grew S. aureus and in each case, SAg genes were detected by PCR. In several cases, the plasma contained biological SAg activity resulting in Vβ specific proliferation of indicator T cellsin vitro. The same specific activity was observed with the supernatant fluids of S. aureus broth cultures from the respective bacterial isolates. This confirms the presence of bio-active toxins in the plasma but did not lead to full blown TSS when T cells were lacking. Thus, S. aureus sepsis/shock can be clinically distinguished from typical TSS, and we suggest that muco-cutaneous manifestations of TSS are the most telling signs of massive T-cell-dependent cytokine release.
KW - Immunodeficiency
KW - Multiple myeloma
KW - Staphylococcus aureus
KW - Superantigen
KW - Toxic shock syndrome
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U2 - 10.1046/j.1365-2249.2002.01805.x
DO - 10.1046/j.1365-2249.2002.01805.x
M3 - Article
C2 - 12033193
AN - SCOPUS:0036243131
SN - 0009-9104
VL - 128
SP - 131
EP - 139
JO - Clinical and Experimental Immunology
JF - Clinical and Experimental Immunology
IS - 1
ER -