Abstract Number: PB0808
Meeting: ISTH 2021 Congress
Background: Catastrophic thrombotic syndromes are characterized by rapid onset of multiple thromboembolic occlusions affecting diverse vascular beds. Patients with hypercoagulability may present with a single thrombosis and subsequently develop progressive thromboses at other sites and develop them rapidly over days to weeks.
Aims: To present the case of fatal thrombotic syndrome in a young boy.
Methods:
| Hb | 99 g/L | D-dimer (N:˂0,5mcg/mL) | 19,7 | Ferritin (N: 7-140 mcg/L) |
382,5 | Circulating immune complexes (N:50-90 IU) |
76,8 |
| RBC | 4,56×1012/L | Anthi thrombin III (N: 80-120%) |
104% | Folate (N:2,3-12 ng/ml) |
9,06 | RF | negative |
| WBC | 9,2×109/L | Protein C (N:70-140%) | 92% | Homocysteine (N:5-15 µmol/L) |
15 | Factor II mutation (20210G>A) | negative |
| PLT | 409×109/L | Protein S (N:57-112%) | 50,9% | Vitamin B12 (271-1170 pg/ml) |
374,1 | Factor V Leiden mutation (1691G>A) | negative |
| CRP | 64,6 mg/L | Plasminogen (N:72-126%) |
92% | beta2-Glykoprotein Ig G |
negative | MTHFR (C677T) mutation | negative |
| aPTT (N:26-36 sec) |
33,4 | Inhibitor plasminogen (N:89-112%) | 91% | Lupus anticoagulant | negative | t-PA mutation | negative |
| PT (N:13-18,8 sec) |
17,2 | Factor VIII (N:50-150%) |
130% | Cardiolipin Ig G | negative | GP1BA (482C>T) |
negative |
| INR | 1,28 | von Willebrand factor (Ag)(N:61-157%) | 220,8% | ANA- Screen | negative | PAI-1 mutation (675 5G>4G) |
heterozygous |
| Fbg | 2,54 g/L | Factor XII (N:50-150%) |
112% | c-ANCA | negative | TNFα (G308A) |
heterozygous |
Clinical presentation and treatment
374,1
| Month | Event | Treatment | |
|---|---|---|---|
| March | Pulmonary embolism. Thrombosis of inferior vena cava, right common iliac, external and internal iliac viens, left common iliac vien (CT). | tPA IV unfractioned heparin infusion (UFH) |
|
| April | Thrombosis of right superficial and deep femoral viens, right tibial, deep plantar and dorsal viens (US doppler) | Continued on UFH | |
| Bilateral thrombosis of common iliac, external and internal iliac viens, femoral, saphena magna viens (US doppler ) | Continued on UFH | ||
| Left renal vien thrombosis (CT) (abdomen and neck pain) | Continued on UFH | ||
| May | Thrombosis of left v.cephalica, v.axilaris, v.subclavia (US doppler) | Warfarin per/os Subcutaneous low molecular weight heparin (LMWH) |
|
| Thrombosis of small vessels of the sigmoid colon with ischemia of the rectum (CT) | Subcutaneous low molecular weight heparin (LMWH) | ||
| June | Extensive pulmonary embolism (CT) | tPA + UFH |
Laboratory investigation
In a previously healthy 8-year-old boy had been developed pain in the left side, legs, breathlessness, increased body temperature to 38,5C. Few days before trauma – falling from a tree. He had no hypercoaugulable disorders and other illnesses, family history was negative too. On examination reduced air entry was identified on the back lower lobes of the lung. His chest x-ray showed bilateral chest infiltrates. CT scan of the chest, abdomen and pelvis showed extensive bilateral pulmonary embolism with bilateral polysegmental lower lobe pneumonia, bilateral hemothorax, areas of thrombosis including inferior vena cava, right common iliac, external and internal iliac viens, left common iliac vien. Clinical presentation and treatment during staying at hospital – Table 1. Regular laboratory testing didn’t show any significant abnormalities – Table 2.
Results: While receiving anticoagulation therapy, there was positive dynamic with 50% of recanalization previous located thromboses (by US doppler) and improving general well-being. But thrombotic syndrome continued to progress with complication by extensive pulmonary emboli. Despite systemic tPA and anticoagulation therapy the patient died.
Conclusions: We have described a fatal case of a young boy who developed multiple acute arterial and venous thrombotic events within 3 month. Catastrophic thrombotic syndrome is an infrequent but potentially fatal presentation of VTE in children. Generally recommended that patients with catastrophic macrovascular thrombotic events must be treated with a long-term antithrombotic therapy.
To cite this abstract in AMA style:
Kashpurenko Y, Vilchevska K, Bakhchyvandzhy I. Catastrophic Thrombotic Syndrome in 8 Year Old Boy: Clinical Case Report [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/catastrophic-thrombotic-syndrome-in-8-year-old-boy-clinical-case-report/. Accessed November 29, 2023.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/catastrophic-thrombotic-syndrome-in-8-year-old-boy-clinical-case-report/