Acute presentations of sickle cell disease complications, often presenting with pain

Potential diagnoses	History	Clinical	Laboratory/imaging/ other testing
Acute systemic illness
Multiorgan failure	Chronic pain, lung disease, or renal insufficiency	Fever, ↑ RR, ↑ HR, BP changes Nonfocal neurologic changes Pain is often severe with rapid organ failure	↓ hemoglobin and platelets ↑ bilirubin, LDH, creatinine Signs of rhabdomyolysis Infiltrate on CXR
Hemolytic transfusion reaction (HTR)	Recent transfusion (within 24 hours for acute HTR; up to one month for delayed HTR) History of alloimmunization	Jaundice, dark urine Fever, ↑ HR	↓ hemoglobin and platelets ↑ bilirubin, LDH Positive Coombs (direct antiglobulin) test Hemoglobinuria
Headache
Stroke	Prior stroke or silent cerebral infarct History of ↑ transcranial Doppler velocities	Headache Focal or non-focal neurologic findings	Brain imaging helpful if positive but may initially be negative Presumptive treatment with simple transfusion is done while obtaining brain imaging if suspicion is high
Brain aneurysm	Usually asymptomatic but may have headache, loss of visual acuity, or facial pain	Usually negative if unruptured but may have cranial neuropathies	Positive MRI or CT angiography
Meningitis*	Fever, meningeal symptoms	Meningismus	↑ WBC count
Migraine	Aura Typical migraine symptoms for that patient	May show focal or nonfocal findings	No diagnostic laboratory test
Chest pain
Acute chest syndrome (ACS) or pneumonia* (indistinguishable)	History of pulmonary disease, prior ACS, asthma, recent infection	↑ RR, ↓ oxygen saturation Children: Fever, cough Adults: Afebrile, severe pain that may initially overshadow the pulmonary symptoms	↓ hemoglobin Infiltrate on CXR helpful if present, but may initially be negative, and absence does not eliminate possibility of ACS
Pulmonary embolism	History of DVT helpful if present but often absent Pregnancy, recent surgery, indwelling catheter, or other hypercoagulable state	↑ RR, ↑ HR ↓ oxygen saturation Extremity pain/swelling	↑ D-dimer Imaging decisions depend on pretest probability and D-dimer Chest imaging and/or extremity imaging may be appropriate
Pulmonary fat embolism	History of pulmonary disease or recent surgery Severe extremity pain	↓ oxygen saturation ↑ RR Nonfocal neurologic symptoms Patients may deteriorate rapidly and develop multi-organ failure	↓ hemoglobin, platelets ↑ nucleated RBCs Infiltrate on CXR Diagnosed by bronchoscopy
Acute coronary syndrome	History of cardiac disease, pulmonary hypertension, or ↑ QT interval Patients with SCD and severe vaso-occlusive pain are increasingly recognized to have acute myocardial ischemia without major vessel disease	Atypical chest pain Radiation to arm(s)	↑ cardiac biomarkers Abnormal EKG
Rib infarct	Recurrent sternal pain	Focal rib tenderness Pain on inspiration Splinting may cause hypoventilation	Negative CXR Positive bone imaging
Abdominal pain
Splenic sequestration	History of splenic or hepatic sequestration Infants with hemoglobin SS; adults with SCD variants	Variable abdominal pain, requires a high index of suspicion Hemodynamic instability, ↑ HR, ↑ BP Splenic enlargement (rapidly enlarging spleen needs to be closely monitored)	↓ hemoglobin, ↓ platelet count ↑ reticulocyte count
Hepatic sequestration	History of splenic or hepatic sequestration History of underlying hepatic disease May be exacerbated by hepatic iron overload, HCV infection, or other causes of liver dysfunction	Right upper quadrant pain and/or tenderness Hemodynamic instability, ↑ HR, ↑ BP Acute hepatomegaly, can progress to acute hepatic failure	↓ hemoglobin ↑ PT, ↑ aPTT hyperbilirubinemia
Gallstones or cholecystitis*	History of gallstones	Acute RUQ pain or tenderness Jaundice Nausea	↑ bilirubin, ↑ transaminases ↑ WBC Positive RUQ ultrasound or other imaging
Renal infarct		Hematuria Back pain and/or flank or CVA tenderness	RBCs on urinalysis Variable ↑ in creatinine Acute papillary necrosis on renal imaging
Ectopic pregnancy	Sexually active reproductive age female	Lower abdominal pain, especially after menses Abnormal uterine bleeding or discharge Nonspecific symptoms (urinary frequency, vaginal discharge) Abdominal and pelvic examinations are often unremarkable if the pregnancy has not ruptured Cervical motion tenderness, adnexal tenderness, or uterine tenderness are common with rupture An adnexal mass may be palpable Excessive pressure on the adnexa should be avoided because it may cause rupture	Positive pregnancy test Diagnosed by serial quantitative HCG testing and transvaginal ultrasound
Pelvic inflammatory disease	Sexually active female	Lower abdominal pain, especially after menses Abnormal uterine bleeding or discharge Nonspecific symptoms (urinary frequency, vaginal discharge) Fever in severe cases Cervical motion tenderness, adnexal tenderness, or uterine tenderness are defining characteristics Purulent vaginal or endocervical discharge	Clinical diagnosis ↑ WBC may be present but is not highly sensitive or specific Additional testing includes pregnancy test, urinalysis, microscopy of vaginal discharge, testing for chlamydia, gonorrhea, HIV, and syphilis Imaging for selected cases to evaluate for complications such as tubo-ovarian abscess or to exclude ectopic pregnancy
Urinary tract infection or pyelonephritis*	Urinary tract infection can precipitate an acute pain episode	Fever Suprapubic tenderness Children: May not report any symptoms Adults: May have urgency, frequency, dysuria, flank pain	Positive urinalysis and culture
Opioid-induced constipation	Recent opioid use, typically within the previous 72 hours Lack of a consistent bowel regimen	Reduced bowel movements
Extremity or bone pain
Acute synovitis or avascular necrosis (AVN) of a joint	History of avascular necrosis	Hip pain Limp Limited range of motion Pain accompanied by joint swelling	Radiography is negative in early stages of AVN MRI may be needed to document findings
Dactylitis	Infant or young child (rarely seen above the age of four years)	Pain with swelling and warmth of the fingers or toes
Gout	Older adults History of renal disease, hypertension, or gout	Joint pain in atypical areas such as phalanges, angles, elbows, wrists Pain accompanied by joint swelling Monoarticular joint pain may be accompanied by more diffuse pain from vaso-occlusion	Variably ↑ creatinine, urate Urate crystals in joint fluid
DVT	Pregnancy, recent surgery, indwelling catheter, or other hypercoagulable state	Pain with leg swelling	↑ D-dimer Compression ultrasonography or other noninvasive testing
Osteomyelitis	History of bone infarction, avascular necrosis, or gastroenteritis	Can present with generalized bone pain Pain accompanied by swelling, tenderness, warmth Variable joint involvement (can be multifocal)	Diagnosis often delayed; often confused with bone infarct (much less common) ↑ WBC Blood, bone, and joint aspirate cultures positive for salmonella, staphylococcus Imaging may show periosteal elevation and/or fluid collection
Generalized/diffuse pain
Neuropathic pain	History of chronic pain and ineffective opioids	Pain described as burning, shooting, or tingling Hyperalgesia to touch or temperature	No diagnostic laboratory tests
Opioid or steroid withdrawal	Recent hospitalization for pain episode Sudden withdrawal of opioid or steroid without tapering	Pain may be severe Agitation, ↑ tremors Gastrointestinal symptoms Sweating	No diagnostic laboratory tests A common cause of readmission When withdrawal is expected, we prefer to use the Clinical Opiate Withdrawal Scale (COWS), an 11-item scale designed to be administered by a clinician

These conditions often present with acute pain in individuals with SCD. Both the pain and the complication should be addressed as rapidly as possible. Refer to UpToDate topics on specific complications and management of acute pain for further information.

SCD: sickle cell disease; RR: respiratory rate; HR: heart rate; BP: blood pressure; LDH: lactate dehydrogenase; HTR: hemolytic transfusion reaction; ACS: acute chest syndrome; RUQ: right upper quadrant; WBC: white blood cell count; CVA: costovertebral angle; DVT: deep vein thrombosis; HCV: hepatitis C virus; HCG: human chorionic gonadotropin; PT: prothrombin time; aPTT: activated partial thromboplastin time; RBC: red blood cell; CXR: chest radiography; MRI: magnetic resonance imaging; CT: computed tomography.
* Individuals with SCD are functionally asplenic and at risk for severe infections, especially with encapsulated organisms.

Graphic 110434 Version 3.0

خرید پکیج

Acute presentations of sickle cell disease complications, often presenting with pain