Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study

Bendapudi, Pavan K.; Hurwitz, Shelley; Fry, Ashley; Marques, Marisa B.; Waldo, Stephen W.; Li, Ang; Sun, Lova; Upadhyay, Vivek; Hamdan, Ayad; Brunner, Andrew M.; Gansner, John M.; Viswanathan, Srinivas R.; Kaufman, Richard M.; Uhl, Lynne; Stowell, Christopher P.; Dzik, Walter H.; Makar, Robert S.

doi:10.1016/s2352-3026(17)30026-1

Cited by 409 publications

(426 citation statements)

References 27 publications

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“…For this reason, useful clinical decision rules to distinguish TTP from pseudo-TMA are essential. When concerning the possibility of TTP, the PLASMIC score, which was recently proposed as a predictive tool for assessing the likelihood of severe ADAMTS13 deficiency in patients with TMA, may be useful 20. The details on PLASMIC score include that one point each awarded for the following: Platelet count <30,000/µL; Evidence of hemolysis (reticulocyte count >2.5%; undetectable haptoglobin, or indirect bili >2.0); No active cancer; No history of solid-organ or stem-cell transplant MCV <90 fl; INR <1.5; Creatinine <2.0 mg/dL.…”

Section: Discussionmentioning

confidence: 99%

Microangiopathic haemolytic anaemia with thrombocytopenia induced by vitamin B12 deficiency long term after gastrectomy

et al. 2018

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Microangiopathic haemolytic anaemia with thrombocytopenia, called pseudo-thrombotic microangiopathy (TMA), is a clinically important complication in patients with vitamin B12 deficiency. We herein present a case of an 80-year-old woman with pseudo-TMA after gastrectomy. She was initially suspected with thrombotic thrombocytopenic purpura based on rapid progression of anaemia with schistocytes and thrombocytopenia; however, her anaemia and thrombocytopenia were improved by vitamin B12 supplementation alone, with a single session of plasma exchange. Vitamin B12 deficiency was finally confirmed by low vitamin B12 levels from the patient's initial blood sample. In addition, normal ADAMTS13 activity was proven, lowering the likelihood of thrombotic thrombocytopenic purpura. Therefore, this patient was diagnosed with pseudo-TMA caused by vitamin B12 deficiency. Pseudo-TMA can occur in patients with vitamin B12 deficiency post-gastrectomy.

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Section: Discussionmentioning

confidence: 99%

Microangiopathic haemolytic anaemia with thrombocytopenia induced by vitamin B12 deficiency long term after gastrectomy

et al. 2018

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“…There is a scoring system that recently was developed to help clinically predict whether the patient has an ADAMST13 level of <10%. This PLASMIC score can be useful in predicting the levels of ADAMST13 to help direct treatment and facilitate early initiation of Rituximab [6]. Our patient received Rituximab on Day 2 of her hospitalization which preceded her drastic improvement.…”

Section: Discussionmentioning

confidence: 99%

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

Ogilvie

Singh

2018

Journal of Community Hospital Internal Medicine Perspectives

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Thrombotic thrombocytopenic purpura (TTP) has a high fatality rate if not caught early and treated with plasmapheresis. When TTP patients present late in their sequelae with neuro symptoms, an elevated lactate dehydrogenase and systemic symptoms, there is a high mortality rate. This report describes the case of a young female who had no significant medical problems and presented to our hospital after several days of hematuria, new onset blurry vision and dizziness. She was found to have thrombocytopenia and microangiopathic hemolytic anemia consistent with TTP and was thus started on plasmapheresis. Her course was further complicated with seizures and development of bilateral basal ganglia infarcts which lead to the need for mechanical ventilation. This was followed by worsening renal functions which was managed with intermittent hemodialysis. To add to her multi-organ failure, she developed shock liver along with demand ischemia evidenced by significant elevations in liver enzymes and troponin leaks, respectively. However, on Day 4 it was fascinating to see the beginning of her recovery pathway. It began with response to simple commands followed by discontinuing invasive ventilation and gradual improvement in her renal functions evidenced by increasing urine output. Soon her platelets started rising consistently and she did not require plasmapheresis or hemodialysis by the time she was discharged. This case highlights the rapid recovery of a young female with new onset TTP which was complicated by involvement and severe damage of more than five different organs but was followed by complete recovery of each organ system.

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“…It is therefore imperative that this association is recognized in clinical practice. Since ADAMTS13 results are not available in real time, a proposed quick guide to determine the likelihood of severely deficient ADAMTS13 activity is PLASMIC score (platelets, lysis, active cancer, stem cell or solid organ transplant, MCV, INR, creatinine): 0-4 score is associated with a low risk of severe ADAMTS13 deficiency, 5 score with intermediate risk and 6-7 scores with high risk [68].…”

Section: Discussionmentioning

confidence: 99%

Interferon-Induced Thrombotic Microangiopathy

Kundra¹,

Andrei²,

Wang³

2018

Thrombocytopenia

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Interferon (IFN) is an effective therapy for multiple disorders. An infrequently reported side effect is thrombotic microangiopathy (TMA): thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We published the first comprehensive review analyzing this association with the following observations: (1) there was a higher incidence of IFN-induced TMA in myeloproliferative disorders (chronic myelogenous leukemia (CML)) than that in nonmalignant disorders (multiple sclerosis (MS), chronic hepatitis C virus infection (HCV)); (2) mean age at diagnosis was 47 years; (3) there was rare association with hairy cell leukemia (HCL), Sezary syndrome (one case each) and no cases reported for polycythemia vera (PV); (4) sex distribution was balanced (exception of higher prevalence in females for MS); (5) TMA was insidious in onset with long incubation periods (average treatment duration 40.4 months); (6) comparative analysis of mean time (months) to onset of TMA ensuing cumulative IFN exposure was: MS 68.6 vs. CML 35.5 vs. HCV 30.4; (7) confirmed TTP (low ADAMTS 13 levels) was associated with the presence of an inhibitor; (8) outcome analysis revealed complete remission in 27 (40%), persistent chronic kidney disease in 28 (42%) and fatality in 12 patients (18%); (9) corticosteroids, plasma exchange (PEX) and rituximab are effective therapies.

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Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study

Cited by 409 publications

References 27 publications

Microangiopathic haemolytic anaemia with thrombocytopenia induced by vitamin B12 deficiency long term after gastrectomy

Microangiopathic haemolytic anaemia with thrombocytopenia induced by vitamin B12 deficiency long term after gastrectomy

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

Interferon-Induced Thrombotic Microangiopathy

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