Case Report

Catastrophic Antiphospholipid Syndrome with Severe Acute Thrombotic Microangiopathy and Hemorrhagic Complications

Table 1

Laboratory data.

AnalyteReference rangeDay 1Day 17

Sodium (mmol/liter)137–145137141
Potassium (mmol/liter)3.6–5.05.93.9
Chloride (mmol/liter)98–107107105
CO2 (mmol/liter)22–301130
Urea nitrogen (mg/dL)7–173388
Creatinine (mg/dL)0.5–1.42.83.3
Glucose (mg/dL)74–199229145
Calcium (mg/dL)8.4–10.27.38.7
Total protein (g/dL)6.3–8.26.35.5
Albumin (g/dL3.9–5.01.82.9
AST (IU/liter)15–465870
ALT (IU/liter)9–522330
LDH (IU/liter)84–2468365235
Bilirubin, total (mg/dL)0.3–1.30.41.9
Bilirubin, indirect (mg/dL)0.2–0.90.31.1
Haptoglobin (mg/dL)30–2008<10
Hemoglobin (g/dL)12–164.210.2
Hematocrit (%)37–4712.829.5
Leukocyte count (×103/μL)4.3–11.025.39.9
 Neutrophils (×103/μL)1.5–7.018.59.3
 Lymphocytes (×103/μL)1.0–3.73.30.3
 Monocytes (×103/μL)0.0–0.71.30.25
 Eosinophils (×103/μL)0.0–0.4N/A0.00
 Basophils (×103/μL)0.0–0.1N/A0.05
Platelet count (×103/μL)140–44089
Urinalysis
 ColorYellowDarkRed
 TurbidityClearCloudyCloudy
 pH4.6–8.05.06.0
 Specific gravity1.001–1.0351.0301.030
 GlucoseNegativeNegative1+
 KetonesNegativeNegativeNegative
 BilirubinNegativeNegative1+
 BloodNegative3+3+
 ProteinNegative2+3+
 NitritesNegativeNegativeNegative
 Leukocyte esteraseNegativePositivePositive
 White blood cells/hpf0–21525>182
 Red blood cells/hpf0–21012>182
Urine protein (mg/dL)5–251001197
Urine creatinine (mg/dL)20–370N/A82
Urine protein: creatinine0.02–0.13N/A14.6
Lupus anticoagulant panelNegativeN/APositive
 Silica clotting test ratio<1.2N/A<1.2
 DRVVT ratio<1.2N/A1.21
 Staclot LA clotting time (sec)<10N/A12.2
Anticardiolipin AbNegativeNegativeNegative
 IgG (U/mL)<100.44.1
 IgM (U/mL)<10N/A3.7
 IgA (U/mL)<87.41.4
Anti- -glycoprotein I AbNegativeNegativeNegative
 IgG (U/mL)<20<9<9
 IgM (U/mL)<20<9<9
ADAMTS-13 activity (%)56–2535859
Antiplatelet factor-4NegativeN/ANegative
CRP (mg/dL)<1.0N/A5.3
ESR (mm/hour)0–20125N/A
Ferritin (ng/mL)8–388397N/A
D-Dimer (ng/mL)0–2304950>5000
FDP (μg/mL)<55–20N/A
Fibrinogen (mg/dL)190–380344228
APTT (sec)24.5–35.723.221.3
PT (sec)10.1–12.612.310.5
C3 (mg/dL)90–1804564
C4 (mg/dL)10–40913
ANA titer<1 : 40Positive1 : 160
Anti-dsDNA Ab (IU/mL)<5.08510
Antichromatin Ab (IU/mL)≤0.9>8.0Positive
Anti-Smith Ab (IU/mL) ≤0.9>8.0Positive
Anti-RNP Ab (IU/mL)≤0.9>8.0Positive
Anti-SSA/Ro Ab (IU/mL)≤0.9>8.0Positive
Anti-SSB/La Ab (IU/mL)≤0.96.0Positive
Anti-Scl-70 Ab (IU/mL)≤0.9≤0.9Negative
Anticentromere Ab (IU/mL)≤0.9≤0.9Negative
Anti-Jo-1 IgGNegativeNegativeNegative
Antiribosomal AbNegativeN/ANegative
Anti-GBM Ab (AI)<1.0<1.0<1.0
ANCA (perinuclear) (AI)<1.0<1.0N/A
ANCA (cytoplasmic) (AI)<1.01.3N/A
Antimyeloperoxidase Ab NegativeN/ANegative
Antiproteinase-3 AbNegativeN/ANegative
Rheumatoid factor (IU/mL)<14N/A<14
CPK (IU/liter)38–234128218
Alk Phos (IU/liter) 38–1268250

Values out of the reference range are in bold. Ab: antibody; ADAMTS-13: a disintegrin and metalloprotease with thrombospondin-1—like domains; AI: antibody index; Alk Phos: alkaline phosphatase; ALT: alanine transaminase; ANA: antinuclear antibodies; ANCA: antineutrophil cytoplasmic antibodies; anti-GBM: antiglomerular basement membrane; anti-dsDNA: antidouble stranded DNA; APTT: activated partial thromboplastin time; AST: aspartate transaminase; CO2: carbon dioxide, CPK: creatine phosphokinase; CRP: C-reactive protein; DRVVT: dilute Russell viper venom time; FDP: fibrin degradation products; LDH: lactate dehydrogenase; N/A: not available; PT: prothrombin time; RNP: ribonucleoproteins; Scl-70: scleroderma-70; sec: seconds.