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]

Overview

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

• Hemolytic uremic syndrome-Thrombotic thrombocytopenic purpura (HUS-TTP)
• Disseminated Intravascular Coagulation (DIC)
• paroxysmal nocturnal hemoglobinuria
• SLE
• HELLP syndrome
• Myeloproliferative 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, splenomegaly	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)									+	Leukopenia, Lymphopenia, Anemia,  Thrombocytopenia	↑	Anti dsDNA, ANA	↑Cr or BUN, ↑ALT or AST, Proteinuria	Interstitial lung disease, Pneumonitis, Pulmonary emboli, Alveolar 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 cyanosis, Hemorrhagic skin infarctions	-	+	Thrombocytopenia, Schistocytes	↑	-	↑D-dimer, aPTT 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	Thalami, brainstem, or cerebellumabnormality		Microthrombosesinclude fibrin thrombi that may occlude the glomerular tufts	Clinical findings coupled with laboratory abnormalities	Hematuria, Proteinuria
		ITP		+/-	-	+	Petechiae	Splenomegaly	+	Anemia, Thrombocytopenia	-	Anti platelet factor 4 antibodies	HIV, ANA	R/O other causes	-	Increased number of normal morphologic megakaryocytes	Clinical findings coupled with thrombocytopenia	Easy bruising, Purpura
		HELLP	-	-	-	-		Hepatomegaly	+	Anemia, Thrombocytopenia, Schistocytes	-	-	↑Bilirubin, ↓Haptoglobin, ↑LDH, ↑Cr	Cortical hypodense areas in the occipital lobes, Diffuse cerebral edema	Ultrasonography shows Poor fetal growth, Oligohydramnios, Abnormal umbilical artery	-	24-hour urine study	Seizure, Edema
	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 [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		+	+	+	+	Petechiae Anal pruritis	Splenomegaly	-	↑WBCs, predominantly neutrophils	+	-	Bone marrow aspiration Trephine biopsy Lumbar puncture Lymph node biopsy.	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	Bruising Epistaxis Bleeding gums Menorrhagia Metrorrhagia Petechiae
Recurrent pregnancy loss	Uterine abnormality
	Chromosomal abnormality
	Hypothyroidism