Lack of muco-cutaneous signs of toxic shock syndrome when T cells are absent:S. aureusshock in immunodeficient adults with multiple myeloma

Abstract

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 there lease 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 trans-plantation. 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 VP specific proliferation of indicator T cells in 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 mucocutaneous manifestations of TSS are the most telling signs of massive T-cell-dependent cytokine release.

Two Cases of Fatal Shock after Transfusion of Platelets Contaminated by Staphylococcus aureus: Role of Superantigenic Toxins

“…However, cases of TSS without cutaneomucous involvement have already been reported and were confirmed by the proliferation of specific Vb-restricted T cells in response to the superantigen [9]. Moreover, desquamation usually occurs 1-2 weeks after onset of TSS, and the patients we describe died 4 and 7 days after the onset of infection [6].…”

“…These cytokines and, especially, TNF-a cause a capillary link syndrome, which generally leads to toxic shock syndrome (TSS) [5]. TSS usually occurs when large amounts of staphylococcal superantigenic toxins are secreted into the circulation, even in immunocompromised patients [6]. Nonmenstrual TSS is now more common than menstrual TSS [5].…”

“…The immunological responses that were observed in the patients after the intravenous bolus of superantigens introduced by these transfusions could have been attenuated by the patients' low lymphocyte and monocyte counts [6]. However, diarrhea, vomiting, disorientation, and myalgia are common manifestations of TSS and have never, to our knowledge, been described as initial signs of transfusion-related S. aureus infection [4,7].…”

Staphylococcus aureusExpress Unique Superantigens Depending on the Tissue Source

“…Eight were obtained at the height of a TSS epidemic in 1983 [3]; 4 were recent isolates; 6 were from cases of menstrual TSS; 6 were from cases of nonmenstrual TSS; and 2 were from immunodeficient patients with atypical presentation of TSS [4]. There was no obvious correlation with these clinical groups in terms of SAG genes found by PCR.…”

Staphylococcal Toxic Shock Syndrome Erythroderma Is Associated with Superantigenicity and Hypersensitivity

“…Although early epidemiologic reports note a few cases lacking rashes [4], Van Lierde et al [10] and Matsuda et al [11] published full clinical descriptions of individual cases. Kamel et al concluded that the absence of rash in three TSS patients, who underwent chemotherapy for multiple myeloma, reflected T cell deficiency [12]. …”