Ann Hematol: Mortality of acquired thrombotic thrombocytopenic purpura in the pre-kapalizumab era

 

Acquired thrombotic thrombocytopenic purpura (aTTP) is caused by the immune- mediated defense of ADAMTS13 metalloproteinases , and then the super-large angiohematoma factor multimers on the surface of the vascular endothelium persist and platelet aggregation increases
.

Although plasma exchange (PEX) and immunosuppressive agents are effective in the treatment of aTTP, many patients still die from the disease

Thrombus immune blood vessels are crucial for the early identification of these patients, because they can benefit from emerging drugs such as capcizizumab, an anti-von Willebrand (vW) nanobody that can prevent platelet aggregation to In small blood vessels, it is still undecided whether capcizizumab can save these patients

Early identification of patients at high risk of death at the first episode of aTTP, paying special attention to the available clinical data,

The researchers searched the Spanish TTP registry for patients who were diagnosed with aTTP during the visit and met complete clinical and follow-up data ( n= 102)
.

These patients were diagnosed between 2004 and 2018, and all received daily PEX and corticosteroid treatment

Diagnose n

Table 1: Clinical data of acquired thrombotic thrombocytopenic purpura

Table 1: Clinical data of acquired thrombotic thrombocytopenic purpura Table 1: Clinical data of acquired thrombotic thrombocytopenic purpura

Eight patients (7.
7%) died between 12 hours and 36 days after presentation, which can be divided into three patterns: death before treatment, early death caused by acute cardiac or neurological events, and late death caused by unresolved aTTP
.

Coma or coma at diagnosis and platelet count <20 × 10 ⁹/L on day 6 of treatment wereindependently associated with increased risk of death

⁹

Table 2: Clinical characteristics of 8 non-surviving patients

Table 2: Clinical characteristics of 8 non-surviving patients Table 2: Clinical characteristics of 8 non-surviving patients

Table 3: Follow-up characteristics of 94 pre-aTTP surviving patients

Table 3: Follow-up characteristics of 94 pre-aTTP surviving patients Table 3 : Follow-up characteristics of 94 pre-aTTP surviving patients

In this study, the overall mortality rate associated with the first episode of aTTP was 8%
.

On the 6th day of treatment, the mortality rate of patients with severe loss of consciousness and failure to achieve platelet response increased

Figure 1: ROC curve analysis of the mortality and prognostic factors of 102 patients with autoimmune thrombotic thrombocytopenic purpura for the first time
.

Coma was assessed at diagnosis; platelet counts were assessed on day 6 except for 2 patients who died earlier

Figure 1: ROC curve analysis of the mortality and prognostic factors of 102 patients with autoimmune thrombotic thrombocytopenic purpura for the first time

Table 4: Outcome of 100 cases of acquired thrombotic thrombocytopenic purpura
.

Two patients who died in a coma were excluded

Table 4: Outcome of 100 cases of acquired thrombotic thrombocytopenic purpura

Severe suppression of consciousness has been identified as a poor prognostic sign of aTTP, indicating the importance of early diagnosis and treatment of aTTP due to intractable and persistent diseases .

The main advantage of this study lies in the homogeneity of the patient series : all cases are frequent episodes of aTTP, the diagnosis of ADAMTS13 activity is absent, there are anti-ADAMTS13 antibodies, and they have received the same PEX and corticosteroid pretreatment
.

The main limitation is the relatively small number of events, which may prevent a more comprehensive analysis of attp-driven mortality patterns
.

The advantage lies in the homogeneity of the patient series.
The main limitation is the relatively small number of events, which may prevent a more comprehensive analysis of attp-driven mortality patterns
.

In general, there is no numbness or coma at the time of diagnosis, and the platelet responds quickly to PEX, which is the most common pattern in patients with first-onset aTTP, and the prognosis is good
.
In contrast, severe conscious depression and persistent thrombocytopenia that are difficult to treat with PEX and immunosuppressants are not common, but the prognosis is poor
.
The latter patient is a litmus test to evaluate the rescue potential of new drugs such as kapumab on the early suppression of microvascular thrombosis
.

 

Original source:

del Río-Garma, J.
, Bobillo, S.
, de la Rubia, J.
et al.
Mortality in acquired thrombotic thrombocytopenic purpura in the pre-caplacizumab era.
Ann Hematol(2021).
https://doi.
org/10.
1007 /s00277-021-04685-8

et al.
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