Serum lactate dehydrogenase and platelet count predict survival in thrombotic thrombocytopenic purpura

Abstract

A significant number (some 20%) of patients with thrombotic thrombocytopenic purpura do not respond to standard therapy and die. We reasoned that early identification of those who are likely to fail standard therapy would allow the rational introduction of other treatment modalities. To this end we prospectively evaluated 27 consecutive patients, examining serum LDH levels and platelet counts as markers of disease activity and as predictors of outcome. All patients were treated, according to a written protocol, with high volume plasma exchange (35 ml/kg), prednisone, aspirin, and persantine. Twenty-one of the 27 patients (78%) are alive following therapy. Initial LDH and platelet values did not distinguish between the survivors and nonsurvivors. However, by day 3 of therapy both LDH levels and platelet counts differed significantly between the two groups. Mean day 3 LDH level for survivors was 364 units/L, and for nonsurvivors, 891 units/L (P < 0.005). Mean day 3 platelet count for survivors was 119,000/microL, and for nonsurvivors, 46,000/microL (P < 0.005). Receiver Operator Characteristic curves were constructed and assessed by calculating the area under the curve. This analysis showed that LDH is able to discriminate survivorship one day earlier than platelet count. Both LDH level and platelet count are excellent predictors of survival, under standard therapy, after 3 days of treatment. Early identification of those at greatest risk will facilitate the early institution and evaluation of alternative methods of treatment, such as splenectomy, intravenous Ig, or Vincristine.

TTP and ADAMTS13: When Is Testing Appropriate?

“…Others reported that platelet count and serum LDH are useful predictors of the response to plasma exchange during the acute episode. 40 In future prospective studies, the potential role of these simpler and inexpensive markers should not be ignored.…”

Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience

“…Severe clinical presentation on diagnosis, including cerebral involvement, renal failure, 7 very low platelet count and profound anemia, was variably associated with a higher mortality rate by some authors 8,9 but were not confirmed by others. 7,[10][11][12][13] Patients at relapse were also reported as having a better disease prognosis. 14 Studies assessed the prognostic value of anti-ADAMTS13 antibodies, and inhibitory anti-ADAMTS13 antibodies were associated with a more severe thrombocytopenia, 15 a delayed response to standard treatment 16,17 or an increased mortality rate.…”

“…Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience Ygal Benhamou, 1 Cyrielle Assié, 1 Pierre-Yves Boelle, 2,3 Marc Buffet, 4 Rana Grillberger, 5 Sandrine Malot, 4 Alain Wynckel, 6 Claire Presne, 7 Gabriel Choukroun, 7 Pascale Poullin, 8 François Provôt, 9 Didier Gruson, 10 Mohamed Hamidou, 11 Dominique Bordessoule, 12 Jacques Pourrat, 13 Jean-Paul Mira, 14 Véronique Le Guern, 15 Claire Pouteil-Noble, 16 Cédric Daubin, 17 Philippe Vanhille, 18 Eric Rondeau, 19 Jean-Bernard Palcoux, 20 Christiane Mousson, 21 Cécile Vigneau, 22 Guy Bonmarchand, 23 Bertrand Guidet, 3,24 Lionel Galicier, 25 Elie Azoulay, 26 Hanspeter Rottensteiner, 5 Agnès Veyradier, 27 and Paul Coppo 4 for the the Thrombotic Microangiopathies Reference Center …”

A dramatic recovery in a patient initially expected to die of TTP & its complications

“…In our particular patient, a steady decline despite early plasmapheresis, Rituximab and methylprednisolone were all warning signs that our patient may in fact have refractory TTP that was not responding to therapy. Refractory TTP has been defined by some studies as TTP in which the platelet count does not double in a period of four days [4,5]. Due to the unpredictable nature of TTP, the typical course, duration of treatment and recovery are not well defined; however, it is known that it may take several days of PEX to see improvement in platelet counts.…”