Differentiating Antiphospholipid syndrome from other diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Feham Tariq, MD [2]


Antiphospholipid syndrome should be differentiated from conditions such as hemolytic uremic syndrome-thrombotic thrombocytopenic purpura (HUS-TTP), disseminated intravascular coagulation (DIC), paroxysmal nocturnal hemoglobinuria, SLE, HELLP syndrome and other myeloproliferative diseases.

Differentiating Antiphospholipid antibody syndrome from other Diseases

Abbreviations: ABG= Arterial blood gas, ANA= Antinuclear antibody, ANP= Atrial natriuretic peptide, ASO= Antistreptolysin O antibody, BNP= Brain natriuretic peptide, CBC= Complete blood count, COPD= Chronic obstructive pulmonary disease, CRP= C-reactive protein, CT= Computed tomography, CXR= Chest X-ray, DVT= Deep vein thrombosis, ESR= Erythrocyte sedimentation rate, HRCT= High Resolution CT, IgE= Immunoglobulin E, LDH= Lactate dehydrogenase, PCWP= Pulmonary capillary wedge pressure, PCR= Polymerase chain reaction, PFT= Pulmonary function test.

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Dyspnea Fever Weight loss Arthralgia Skin manifestation Organomegaly Focal neurological disorder CBC ESR Antibodies Other CT scan Other
Antiphospholipid syndrome + + - - Livedo reticularis, palpable purpura, leg ulcers, raynaud phenomena hepatomegaly,


Stroke, transient ischemic attack Schistocytes on peripheral smear,Thrombocytopenia, Hemolytic anemia Lupus anticoagulant,anticardiolipin antibodies - Stroke,

Pulmonary embolism, Budd-Chiari syndrome

Doppler ultrasonography for recurrent DVT Noninflammatory bland thrombosis without perivascular inflammation Hx of thrombosis and antiphospholipid antibodies Miscarriage, Pulmonary hypertension
Systemic lupus erythematosus (SLE) +  LeukopeniaLymphopeniaAnemia,  Thrombocytopenia Anti dsDNAANA Cr or BUN,

ALT or ASTProteinuria

Interstitial lung disease, PneumonitisPulmonary emboliAlveolar hemorrhage Central nervous system (CNS) lupuswhite-matter changes in MRI Staging lupus nephritis Anti-dsDNA antibody test Skin rashes or photosensitivity
Thrombosis Thrombophilia DIC + + - +/-  Acral cyanosisHemorrhagic skin infarctions - + ThrombocytopeniaSchistocytes - D-dimeraPTT and PT Intracranial hemorrhage - Ischemia and necrosis due to fibrin deposition in small and medium-sized vessels Clinical findings coupled with laboratory abnormalities -
HUS + + - + Liver/spleen swelling + Anemia,

Thrombocytopenia, Reticulocytosis

ADAMTS13 Increased lactate dehydrogenase Thalamibrainstem, or cerebellumabnormality Microthrombosesinclude fibrin thrombi that may occlude the glomerular tufts Clinical findings coupled with laboratory abnormalities HematuriaProteinuria 
ITP +/- - + Petechiae Splenomegaly + AnemiaThrombocytopenia - Anti platelet factor 4 antibodies HIVANA R/O other causes - Increased number of normal morphologic megakaryocytes Clinical findings coupled with thrombocytopenia Easy bruisingPurpura
HELLP - - - - Hepatomegaly + AnemiaThrombocytopeniaSchistocytes - - Bilirubin, ↓Haptoglobin, ↑LDH, ↑Cr Cortical hypodense areas in the occipital lobes, Diffuse cerebral edema Ultrasonography shows Poor fetal growthOligohydramnios, Abnormal umbilical artery  - 24-hour urine study  SeizureEdema
Paroxysmal nocturnal hemoglobinuria (PNH) + + - +/- Anemia, reticulocytosis,

Increased lactate dehydrogenase (LDH) and bilirubin

- Direct antiglobulin (Coombs) testing (DAT) Increased haptoglobin - - Bone marrow biopsy shows normocellular or hypercellular bone marrow with erythroid hyperplasia Flow cytometry
Heparin-induced thrombocytopenia (HIT) + + - -
  • Redness of skin
  • Purpura
  • Bleeding from the skin
- + Thrombocytopenia + Anti-heparin-PF4 IgG
  • Serotonin release assay (SRA)
  • Heparin induce platelet aggregation (HIPA) assays
  • Solid-phase immunoassay (SPI) (H-PF4 enzyme-linked immunosorbent assay [[[Enzyme linked immunosorbent assay (ELISA)|ELISA]]])
  • Particle gel immunoassay (PIFA)
Stroke, pulmonary embolism, mesenteric ischemia, intraabdominal or retroperitoneal bleeding, or acute limb ischemia. - Schistocytes Serotonin release assay Limb gangrene

Skin necrosis

Myeloproliferative diseases + + + + Splenomegaly - WBCs, predominantly neutrophils + -
  • Enlarged lymph nodes
  • Splenomegaly
  • Splanchnic venous thrombosis
 PET scan- metastasis in bone marrow   Terminal myeloid cell expansion  Philedelphia chromosome by PCR or BCR/ABL by FISH
Recurrent pregnancy loss Uterine abnormality - - - - - - - - - - - - - - Ultrasound -
Chromosomal abnormality - -/+ - - - - - - - - - - - - Karyotyping -
Hypothyroidism - -/+ - - Myxedema

Dry skin

- - - + - - -
  • Normal/ low TSH
  • Assays for anti-TPO and anti-Tg Ab
  • Dry skin
  • Hair loss


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