Differentiating Antiphospholipid syndrome from other diseases: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{ | [[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Antiphospholipid_syndrome]] | ||
{{ | {{CMG}}{{AE}}{{FT}} | ||
==Overview== | ==Overview== | ||
Antiphospholipid syndrome | Antiphospholipid syndrome should be differentiated from conditions such as [[hemolytic uremic syndrome]]-[[thrombotic thrombocytopenic purpura]] (HUS-TTP), [[disseminated intravascular coagulation]] (DIC), p[[Vasculitis|aroxysmal nocturnal hemoglobinuria]], [[SLE]], [[Cryoglobulinemia|HELLP syndrome]] and other [[Myeloproliferative neoplasm|myeloproliferative]] diseases. | ||
==Differentiating Antiphospholipid antibody syndrome from other Diseases== | ==Differentiating Antiphospholipid antibody syndrome from other Diseases== | ||
* [[Hemolytic uremic syndrome]]-[[Thrombotic thrombocytopenic purpura]] (HUS-TTP) | * [[Hemolytic uremic syndrome]]-[[Thrombotic thrombocytopenic purpura]] (HUS-TTP) | ||
* [[Disseminated Intravascular Coagulation]] (DIC) | * [[Disseminated Intravascular Coagulation]] (DIC) | ||
* [[Vasculitis]] | * [[Vasculitis|paroxysmal nocturnal hemoglobinuria]] | ||
* | * [[SLE]] | ||
* [[ | * [[Cryoglobulinemia|HELLP syndrome]] | ||
* [[ | * [[Myeloproliferative neoplasm|Myeloproliferative]] diseases | ||
<span style="font-size:85%">'''Abbreviations:''' | |||
ABG= [[Arterial blood gas]], ANA= [[Antinuclear antibody]], ANP= [[Atrial natriuretic peptide]], ASO= [[Antistreptolysin O|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]].</span> | |||
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center" | |||
! colspan="3" rowspan="4" |Diseases | |||
| colspan="7" rowspan="1" style="background:#4479BA; color: #FFFFFF;" align="center" |'''Clinical manifestations''' | |||
! colspan="7" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Para-clinical findings | |||
| colspan="1" rowspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |'''Gold standard''' | |||
! rowspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Additional findings | |||
|- | |||
| colspan="4" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |'''Symptoms''' | |||
! colspan="3" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" | Physical examination | |||
|- | |||
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab Findings | |||
! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging | |||
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Histopathology | |||
|- | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea | |||
! colspan="1" rowspan="1" style="background:#4479BA; color: #FFFFFF;" align="center" | Fever | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |Weight loss | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |Arthralgia | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |Skin manifestation | |||
! colspan="1" rowspan="1" style="background:#4479BA; color: #FFFFFF;" align="center" |Organomegaly | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |Focal neurological disorder | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |CBC | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |ESR | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |Antibodies | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |Other | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |CT scan | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" |Other | |||
|- | |||
! colspan="3" |[[Antiphospholipid syndrome]] | |||
!+ | |||
!+ | |||
!- | |||
!- | |||
![[Livedoid vasculitis|Livedo]] reticularis, palpable [[purpura]], leg [[Ulcer|ulcers]], [[Raynaud's phenomenon|raynaud]] phenomena | |||
![[hepatomegaly]], | |||
[[splenomegaly]] | |||
![[Stroke]], [[Transient ischemic attack|transient]] ischemic attack | |||
![[Schistocytes]] on peripheral smear,[[Thrombocytopenia]], [[Hemolytic anemia]] | |||
!↑ | |||
![[Lupus]] [[anticoagulant]],[[Anti-cardiolipin antibodies|anticardiolipin]] antibodies | |||
!- | |||
![[Stroke]], | |||
[[Pulmonary embolism]], [[Budd-Chiari syndrome]] | |||
![[Doppler ultrasound|Doppler]] ultrasonography for recurrent [[Deep vein thrombosis|DVT]] | |||
!Noninflammatory bland [[thrombosis]] without perivascular [[inflammation]] | |||
!Hx of [[thrombosis]] and [[antiphospholipid antibodies]] | |||
![[Miscarriage]], [[Pulmonary hypertension]] | |||
|- | |||
! colspan="3" |[[Systemic lupus erythematosus|Systemic lupus erythematosus (SLE)]] | |||
! | |||
! | |||
! | |||
! | |||
! | |||
! | |||
!+ | |||
! [[Leukopenia]], [[Lymphopenia]], [[Anemia]], [[Thrombocytopenia]] | |||
!↑ | |||
![[Anti-dsDNA antibody|Anti dsDNA]], [[ANA]] | |||
!↑[[Creatinine|Cr]] or [[Blood urea nitrogen|BUN]], | |||
↑[[ALT]] or [[AST]], [[Proteinuria]] | |||
!Interstitial lung disease, [[Pneumonitis]], [[Pulmonary emboli]], [[Hemorrhage|Alveolar hemorrhage]] | |||
![[Central nervous system|Central nervous system (CNS)]] [[Systemic lupus erythematosus|lupus]]<nowiki/>white-matter changes in [[MRI]] | |||
!Staging [[lupus nephritis]] | |||
![[Anti-dsDNA antibody|Anti-dsDNA antibody test]] | |||
![[Skin rashes]] or [[photosensitivity]] | |||
|- | |||
! rowspan="7" |[[Thrombosis]] | |||
! rowspan="4" |[[Thrombophilia]] | |||
![[Disseminated intravascular coagulation|DIC]] | |||
!+ | |||
!+ | |||
!- | |||
!+/- | |||
! [[Cyanosis|Acral cyanosis]], [[Hemorrhagic]] skin [[Infarction|infarctions]] | |||
!- | |||
!+ | |||
![[Thrombocytopenia]], [[Schistocytes]] | |||
!↑ | |||
!- | |||
!↑[[D-dimer]], [[aPTT]] and [[PT]] | |||
![[Intracranial hemorrhage]] | |||
!- | |||
![[Ischemia]] and [[necrosis]] due to [[Fibrin|fibrin deposition]] in small and medium-sized [[vessels]] | |||
!Clinical findings coupled with [[Medical laboratory|laboratory abnormalities]] | |||
!- | |||
|- | |||
![[ITP|HUS]] | |||
!+ | |||
!+ | |||
!- | |||
!+ | |||
! | |||
![[Liver]]/[[spleen]] swelling | |||
!+ | |||
![[Anemia]], | |||
[[Thrombocytopenia]], [[Reticulocyte|Reticulocytosis]] | |||
!↑ | |||
![[ADAMTS13]] | |||
!Increased [[lactate dehydrogenase]] | |||
![[Thalami]], [[brainstem]], or [[cerebellum]]<nowiki/>abnormality | |||
! | |||
![[Thromboses|Microthromboses]]<nowiki/>include [[Fibrin|fibrin thrombi]] that may occlude the [[glomerular]] tufts | |||
!Clinical findings coupled with [[Medical laboratory|laboratory abnormalities]] | |||
![[Hematuria]], [[Proteinuria]] | |||
|- | |||
![[Idiopathic thrombocytopenic purpura|ITP]] | |||
! | |||
!+/- | |||
!- | |||
!+ | |||
![[Petechia|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 syndrome|HELLP]] | |||
!- | |||
!- | |||
!- | |||
!- | |||
! | |||
![[Hepatomegaly]] | |||
!+ | |||
![[Anemia]], [[Thrombocytopenia]], [[Schistocytes]] | |||
!- | |||
!- | |||
!↑[[Bilirubin]], ↓[[Haptoglobin]], ↑[[Lactate dehydrogenase|LDH]], ↑[[Creatinine|Cr]] | |||
!Cortical hypodense areas in the [[Occipital lobe|occipital lobes]], Diffuse [[cerebral edema]] | |||
!Ultrasonography shows [[Fetal growth retardation|Poor fetal growth]], [[Oligohydramnios]], Abnormal [[umbilical artery]] | |||
!- | |||
![[Urinalysis|24-hour urine study]] | |||
![[Seizure]], [[Edema]] | |||
|- | |||
! colspan="2" |[[Paroxysmal nocturnal hemoglobinuria|Paroxysmal nocturnal hemoglobinuria (PNH)]] | |||
!+ | |||
!+ | |||
!- | |||
!+/- | |||
! | |||
! | |||
! | |||
![[Anemia]], [[Reticulocyte|reticulocytosis]], | |||
Increased [[lactate]] [[dehydrogenase]] (LDH) and [[bilirubin]] | |||
!- | |||
!Direct [[Coombs test|antiglobulin]] (Coombs) testing (DAT) | |||
!Increased [[haptoglobin]] | |||
!- | |||
!- | |||
!Bone marrow biopsy shows normocellular or hypercellular bone marrow with [[Red blood cell|erythroid]] [[hyperplasia]] | |||
![[Flow cytometry]] | |||
! | |||
|- | |||
! colspan="2" |[[Heparin-induced thrombocytopenia|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 [[Retroperitoneum|retroperitoneal]] bleeding, or acute limb [[ischemia]]. | |||
!- | |||
![[Schistocytes]] | |||
!Serotonin release assay | |||
!Limb gangrene | |||
Skin necrosis | |||
|- | |||
! colspan="2" |[[Myeloproliferative disease|Myeloproliferative diseases]] | |||
!+ | |||
!+ | |||
!+ | |||
!+ | |||
! | |||
* [[Petechiae]] | |||
* Anal pruritis | |||
![[Splenomegaly]] | |||
!- | |||
!↑[[White blood cells|WBCs]], predominantly [[Neutrophil|neutrophils]] | |||
!+ | |||
!- | |||
! | |||
* [[Bone marrow aspiration]] | |||
* [[Biopsy|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]] | |||
|- | |||
! rowspan="3" |[[Recurrent pregnancy loss]] | |||
! colspan="2" |[[Uterine]] abnormality | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
![[Ultrasound]] | |||
!- | |||
|- | |||
! colspan="2" |[[Chromosomal abnormality]] | |||
!- | |||
!-/+ | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
!- | |||
![[Karyotyping]] | |||
!- | |||
|- | |||
! colspan="2" |[[Hypothyroidism]] | |||
!- | |||
!-/+ | |||
!- | |||
!- | |||
![[Myxedema]] | |||
Dry skin | |||
!- | |||
!- | |||
!- | |||
!+ | |||
!- | |||
!- | |||
!- | |||
! | |||
* Low [[T3]],[[T4]] | |||
* Normal/ low [[Thyroid-stimulating hormone|TSH]] | |||
* Rest of [[pituitary]] hormone levels WNL | |||
!- | |||
! | |||
* Assays for anti-TPO and anti-Tg Ab | |||
! | |||
* Dry skin | |||
* [[Bradycardia]] | |||
* Hair loss | |||
* [[Myxedema]] | |||
* Delayed relaxation phase of deep [[Tendon reflex|tendon reflexes]] | |||
|- | |||
|} | |||
==References== | ==References== |
Latest revision as of 20:32, 20 February 2019
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, | Stroke, transient ischemic attack | Schistocytes on peripheral smear,Thrombocytopenia, Hemolytic anemia | ↑ | Lupus anticoagulant,anticardiolipin antibodies | - | Stroke, | 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, | ↑ | 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) | + | + | - | - |
|
- | + | Thrombocytopenia | + | Anti-heparin-PF4 IgG |
|
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 | + | - |
|
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 |
- | - | - | + | - | - | - |
|
- |
|
|