J Med Cases

Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc

Journal website https://www.journalmc.org

Case Report

Volume 12, Number 7, July 2021, pages 262-266

An Exceedingly Rare Case of Antithrombin III Deficiency and Catastrophic Antiphospholipid-Like Disease

Figure 1. (a) Axial CT of the abdomen demonstrating splenic and hepatic infarcts. (b) Axial CT of the abdomen demonstrating filling defect in the descending aorta. (c) Axial CT of the left lower extremity demonstrating filling defect in the femoral artery. (d) Axial CT of the head demonstrating right frontal and occipital infarcts. CT: computed tomography.

**Table 1.** The Revised Sapporo Classification Criteria for Diagnosis of APS [4]
Antiphospholipid syndrome is present if at least one of the following clinical and one of the laboratory criteria are met. APS: antiphospholipid syndrome; IgG: immunoglobulin G; IgM: immunoglobulin M.
Clinical criteria
1) Vascular thrombosis: At least one clinical episodes of arterial, venous, or small vessel thrombosis in any tissue or organ. Thrombosis must be confirmed by objective validated criteria from imaging or histopathology. For histopathologic confirmation, thrombosis should be present without significant evidence of inflammation of the surrounding vascular wall.
2) Pregnancy morbidity
	A: At least one unexplained death of a morphologically normal fetus at or beyond week 10 of gestation, with normal fetal morphology documented by ultrasound or by direct examination of the fetus; or
	B: At least one premature birth of a morphologically normal neonate before the 34th week of gestation because of: 1) eclampsia or severe preeclampsia or 2) recognized features of placental insufficiency; or
	C: At least three unexplained consecutive spontaneous abortions before week 10 of gestation, with maternal anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded.
Laboratory criteria
1) Lupus anticoagulant present in plasma on at least two occasions (at least 12 weeks apart).
2) Anticardiolipin of IgG and/or IgM isotype in serum or plasma, present in medium or high titer (> 40 GPL or MPL or > 99th percentile) on at least two occasions (at least 12 weeks apart).
3) Anti-beta-2 glycoprotein I antibody of IgG and/or IgM isotype in serum or plasma, present in high titer (> 99th percentile) on at least two occasions (at least 12 weeks apart).

Table 1. The Revised Sapporo Classification Criteria for Diagnosis of APS [4]

Antiphospholipid syndrome is present if at least one of the following clinical and one of the laboratory criteria are met. APS: antiphospholipid syndrome; IgG: immunoglobulin G; IgM: immunoglobulin M.

Clinical criteria

1) Vascular thrombosis: At least one clinical episodes of arterial, venous, or small vessel thrombosis in any tissue or organ. Thrombosis must be confirmed by objective validated criteria from imaging or histopathology. For histopathologic confirmation, thrombosis should be present without significant evidence of inflammation of the surrounding vascular wall.

2) Pregnancy morbidity

A: At least one unexplained death of a morphologically normal fetus at or beyond week 10 of gestation, with normal fetal morphology documented by ultrasound or by direct examination of the fetus; or

B: At least one premature birth of a morphologically normal neonate before the 34th week of gestation because of: 1) eclampsia or severe preeclampsia or 2) recognized features of placental insufficiency; or

C: At least three unexplained consecutive spontaneous abortions before week 10 of gestation, with maternal anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded.

Laboratory criteria

1) Lupus anticoagulant present in plasma on at least two occasions (at least 12 weeks apart).

2) Anticardiolipin of IgG and/or IgM isotype in serum or plasma, present in medium or high titer (> 40 GPL or MPL or > 99th percentile) on at least two occasions (at least 12 weeks apart).

3) Anti-beta-2 glycoprotein I antibody of IgG and/or IgM isotype in serum or plasma, present in high titer (> 99th percentile) on at least two occasions (at least 12 weeks apart).