Hemeonc Flashcards
anticoagulants and their reversal agents
-vitamin K does not work fast
-PCC! or FFP + vitamin K for immediate reversal
when do we watch and wait vs immediate reversal
-Major bleeding in a critical site:
-Intracranial bleed
-Intraperitoneal bleed
-Retroperitoneal bleed
-Pericardial tamponade (from bleed)
-Hemothorax
-GI bleeding
-Intramuscular (e.g. compartment syndrome, limb loss)
-Intraarticular
-things you cant apply pressure to
-Hemodynamic instability
-SBP <90 mmHg or decrease >40mmHg
-Syncope
-Hgb drop of ≥ 2 g/dL from bleed
-Requiring ≥2 U PRBCs
-Likely okay to watch and wait:
-Menstrual bleed
-Epistaxis
-Mild GI bleeding
-Cutaneous bleeding
A 3-year old boy presents with oozing blood from his mouth after falling and striking his face on the floor 6 hours ago and he is still bleeding. His mother states that he tends to bleed for prolonged periods from his immunization sites, but there is no history of unusual bruising or hematomas. There is no known family history of a bleeding disorder.
Vitals signs: HR 128, RR 20, O2sat 100% on room air.
General: Alert, in no apparent distress
HEENT: Two small lacerations on the inside of lower lip, oozing blood.
Skin: Petechiae is noted on lower extremities.
Remainder of exam within normal limits (notably, no bruises or joint swelling).
von willebrand ds (dont need to know details)
-Inherited coagulopathy
-Caused by too little von Willebrand factor, or, defective vwf
-no clots are made
-Type 1 vWD is a quantitative defect
-MC (70%)
-Autosomal dominant
-Mild bleeding
-Type 2 vWD is a qualitative defect
-4 subtypes with bleeding tendency variation
-Type 3 vWD is a quantitative defect
-Most severe form where the body produces NO vWF!
-Mucocutaneous bleeding:
-Common: Epistaxis, bruising, menorrhagia
-Type 3: Hemarthrosis and GI bleeds
-Labs will show normal platelets!!! (but will have dysfunction) and elevated PTT!!!!
-Specific vWF labs can be sent for outpatient specialist referral but are outside ED scope
-affects the intrinsic pathway
von willebrand ds tx
-!!Desmopressin (DDVAP)
-Promotes release of vWF from endothelium
-Works well for type 1, okay for type 2 (bc type 3 has NO vWF)
-IV or nasal spray
-Cryoprecipitate
-Contains vWF and factor VIII
-Tranexamic acid (TXA) – antifibrinolytic
-Von Willebrand concentrate
hemophilia A and B
-A 5-day-old male newborn presents with prolonged bleeding after circumcision.
-A 27-year-old male with a history of hemophilia A presents with complaints of headache and nausea after bumping his head against an open cabinet door.
-Consider new hemophilia in unprovoked soft tissue bleeding or hematoma, hematuria, hemarthrosis, neonates with persistent oozing stump
-Hemophilia A: Inherited or acquired deficiency of factor VIII
-Hemophilia B: Inherited or acquired deficiency of factor IX
-Hemophilia C: Inherited deficiency of factor XI
-Do not be falsely reassured by a minor mechanism of trauma and normal examination, especially in cases of head trauma
hemophilia A and B tx
-Suspected bleeding, or trauma, with known hemophilia?
-Factor repletion BEFORE going to CT scan
-Hemophilia A:
-!!Factor VIII : 25U/kg for moderate, 50U/kg for severe/CNS bleeding
-Cryoprecipitate if no factor immediately available (has factor VIII)
-Hemophilia B:
-!!Factor IX : 100U/kg for severe bleeding
-FFP if no factor is immediately available
-Hemophilia A patients are at high risk for DELAYED bleeds. If someone with hemophilia A was in an MVA with head injury, give the Factor VIII regardless of a negative CT scan
sickle cell ds
-Sickle gene found in 8-10% on AA
-Homozygous HgS = most serious form
-Sxs do not appear until 4mo of age when HgF is replaced by the abnormal HbA
-Short RBC half life = chronic anemia [normal is 12-18]
-they live in chronic anemia- hmg 6-8
-Spleen is often the first organ affected by a sickling cell
-Recurrent splenic infarcts -> decline in splenic function by 4-12 months of age -> susceptibility to encapsulated bacteria
-Triggers:
-infection
-dehydration
-stress
-extreme temperatures
acute complications of sickle cell ds
-Hypotension / tachycardia
-!!Sepsis
-!!Splenic sequestration
-!!Aplastic crisis
-Neuro changes
-Stroke / TIA
-CRAO
-Severe pain
-!Acute chest syndrome
-!Priapism
-Bone or joint pain:
-!!Vasoocclusive crisis (VOC) * dx of exclusion
-Dactylitis
-Septic arthritis
-AVN / bone infarcts
-Osteomyelitis
-Abdominal pain
-!Splenic sequestration
-Acute cholecystitis
-Acute sickle hepatopathy (heptic crisis, hepatic sequestration, intrahepatic cholestasis)
-Shortness of breath
-!Acute chest syndrome
-Pulmonary embolism
-Infection
-Acute anemia
-!Aplastic crisis
-!Splenic or hepatic sequestration
-!Acute chest syndrome
-Sepsis
-Hemorrhage
-Renal papillary necrosis
-Other acute blood loss
-Fever
-!Acute chest syndrome
-!Vasoocclusive crisis
-Delayed transfusion reaction
-!Infection / Sepsis (think encapsulated organisms)
-Renal symptoms
-Renal papillary necrosis -> hematuria
-Glomerulonephritis
-AKI
sickle cell approach
-ABCDE
-Maintain sats >95%
-Early and aggressive pain control
-Utilize incentive spirometer -> encourges deep breath despite pain -> prevents acute chest syndrome
-Lab tests:
-CBC w/ diff
-BMP w/ LFTs
-!!RETICULOCYTE count- would be higher in these pts normally -> if aplastic crisis it will be lower
-Lactate dehydrogenase (LDH)- if its high it can mean hemolysis
-Consider: CXR (fever, respiratory symptoms, chest pain), Viral swab, blood culture (if fever), UA, Urine culture, CSF analysis or synovial fluid analysis in the setting of fever
-ALWAYS EVALUATE:
-Cardiopulmonary symptoms
-Hemoglobin level
-Reticulocyte count
-Biomarkers, imaging and laboratory testing (such as elevated reticulocyte count) are unreliable indicators of pain severity
vaso occlusive events
-MC manifestation of SCD
-Can occur in any system:
-pulmonary arterial tree (CP, SOB)
-Coronary arteries (STEMI)
-CNS (HA, strokes)
-MSK (dactylitis, bone pain, VTE)
-Kidneys (CKD/ESRD, Hematuria etc)
-will often have mild !fever and leukocytosis!
-Can lead to !bony infarctions!
-Increased WBCs
-Fat embolism -> acute respiratory distress, petechia, AMS, renal/liver failure
-Vasoocclusive Pain crisis- MC pain locations: long bones, lower back
-Stroke
-Acute chest syndrome
-Papillary necrosis
-Avascular necrosis-DDX: Osteomyelitis
-Ulcers
-Dactylitis:
-Painful and swollen hands/feet
-Usually < 2 years old
-Usually first presentation
vaso-occlusive pain crisis (VOC) tx
-Analgesia
-Prompt and proactive
-Re-evaluate q15-30 minutes
-Titrate meds to effect
-Consider PCA pump
-Add Benadryl q4-6 hrs for itching
-Gentle hydration to euvolemia:
-D5 ½NS @ 1.0-1.5x maintenance (hypotonic)
-Do NOT IV bolus , avoid overhydration -> pulmonary edema
-Maintain SPO2 at least 95%
-Remember: pain crisis can overlap with other complications
-Dispo:
-Admit if required ≥2 doses of narcotics
-Discharge if pain controlled, with PO meds
-Keep ddx broad: always question the patient regarding “normal patterns” of previous crises
vaso-oclusive pain crisis myths
-all pts with SCD are drug seeking
-<5% have a true opioid addiction
-it is true that they develop analgesic tolerance
-pts cannot have normal vitals if they are in pain
-vitals are UNRELIABLE predictor of pain
-if pt is having pain, then they are having a pain crisis right??
-VOC is dx of EXCLUSION
-avoid anchoring, look for other life threatening patholgies
A 35 year old otherwise healthy woman with a known history of sickle cell disease comes in a week after her last uneventful pain crisis with a 2 day history of non-productive cough and gradual onset non-pleuritic severe central chest pain radiating to both shoulders. She admits to being short of breath on exertion.
Her vitals show that she is bit tachycardic and dyspneic with a normal blood pressure, an oxygen saturation of 92% on room air and a borderline Temp of 100.1F (37.8).
Her cardiovascular exam is essentially normal and her chest is clear. The chest x-ray, however, shows multilobar infiltrates.
acute chest syndrome
vaso-occlusive event: acute chest syndrome
-Can occur anytime after a VOC
-Treat before it occurs: Incentive spirometry, Analgesia
-!!MCC of morbidity and mortality in SCD (10%)
-!!!!Fever, cough!, chest pain!, dyspnea, hypoxia!, wheeze, rib/sternum pain
-Tachypneic, rales, wheezing, effusion, low O2
-CXR: !New pulmonary opacity!
-multilobar infiltrates
-Management:
-!Antibiotics - make sure ischemia tissue isnt getting infected
-Pain control
-Supplemental oxygen maintain SPO2>95%
-Incentive spirometry
-Consider transfusion guided by hematologist
-!Admit due to high chance of respiratory failure
which pts with severe acute chest syndrome require emergency exchange transfusion
-In mild cases, reasonable to do 1-2 units PRBC simple transfusion
-Severe cases may require exchange transfusion:
-Patients with a history of severe acute chest syndrome requiring exchange
-Patients with diffuse acute chest X-ray findings
-Oxygenation continues to worsen despite simple transfusion
-Hypoxemia is particularly severe (for example, below 85%)
-Evidence of other organ systems being involved, especially multiorgan failure following. There is a possible association with declining platelet count at the time of presentation
other vaso-occlusive events
-Strokes
-Increased risk of stroke (hemorrhagic and ischemic)
-Activate stroke team
-Simple or exchange transfusion within 2 hours of symptom onset (rather than TPA)
-Acute hepatic crisis and/or sequestration
-Hepatic ischemia from vasoocoulsive crisis
-RUQ pain, jaundice, hepatomegaly, transaminitis
-Acute splenic sequestration (read more later!)
-Splenic vasoocclusion traps large amount of RBCs in spleen -> splenomegaly
-LUQ pain, splenomegaly, hypotension/shock, anemia (Hgb drop ≥2g/dL) with reticulocytosis
-Priapism
-Low flow priapism
-Needle aspiration of blood from the corpora cavernosa, followed by intercavernosal injection of 1ml aliquots (up to 3 ml) of 100-500ug/ml phenylephrine
-Complication: Impotence
A 24 year old man comes back to your ED for the 24thtime, but this time he’s rushed into your resuscitation room as his BP is in the boots. EMS tells you that he had been feeling generally unwell for the past 24hrs with fatigue, SOB and a low grade fever. His girlfriend called 911 when he had a syncopal episode trying to get out of bed.
On physical exam his GCS is 13, his heart rate is 130, blood pressure 70/40, oxygen saturation 90% on a non-rebreather and he has crackles bilaterally to the mid-scapula.
You do your usual resuscitation magic to stabilize him, send off sepsis blood work and Troponins and a while later his hemoglobin comes back at 4.5 – Ahugedrop from his previous 7. A quick rectal exam reveals normal stool with no melena or hematochezia.
You think to yourself, why in the world is this Sickle Cell patient suddenly in shock with severe acute anemia?
acute anemia: sickle cell
-Baseline hemoglobin is usually 6-8 g/dL for HbSS
-Acute anemia if drop of HGB ≥ 2g/dL from baseline or Hgb < 6 g/dL
-Evaluate for potential causes of acute anemia in SCD:
-Aplastic crisis *
-Splenic sequestration *
-Hepatic sequestration *
-Acute chest syndrome
-Delayed hemolytic transfusion reaction
-Infection
-Sepsis
-Other (non-SCD related) blood loss
-!All patients with acute anemia should be admitted
sickle cell: acute anemia: aplastic crisis
-7-year-old boy with SCD-SS is brought in by his parents for the presence of fever. Following laboratory testing, the hemoglobin (Hgb) level is much lower than baseline and the reticulocyte count is <1.
-Severe and sudden drop in hemoglobin AND reticulocytes
-Associated with parvovirus b19
-Fever, pallor, fatigue, lethargy, shock dyspnea
-Labs: Pancytopenia
-Anemia with HGB ~3-6 g/dL (normal 12-16g/dL)
-!!Reticulocytopenia (as low as 0%) (normal 0.5-2.5%)
-most important aspect of tx: simple RBC transfusion!!
-Dispo: Admit! until bone marrow production is resumed
acute anemia: splenic sequestration
-Splenic vasoocclusion and entrapment of large amounts of blood in the spleen that can in severe cases cause shock
-Children! < 5 years old mostly (they still have their spleens…)
-LUQ pain, splenomegaly!
-Anemia (fatigue, pallor)
-Fever
-Shock: HR, RR, BP, poor perfusion
-Supportive findings:
-!Acute anemia (drop HGB of ≥2g/dL from baseline)
-!!Normal or ↑ reticulocytosis
-!Thrombocytopenia (↓ platelets) (d/t trapping)
-Ranges from minor to major, can progress to shock and death within hours
-Supportive management: Fluid resuscitation, cautious pRBC transfusion, elective splenectomy
-Admit
Acute hemoglobin drop + low reticulocyte count =
Acute hemoglobin drop + normal/high retic + abdominal pain =
Sickle cell disease + hypoxemia =
Painful and swollen hands and fingers =
Management for patient with sickle cell crisis and focal neuro deficits consistent with stroke =
Most common cause of osteomyelitis in sickle cell?
-aplastic crisis e
-splenic sequestration
-acute chest syndrome
-dactylitis
-transfusion
-salmonella
A 45-year-old female on coumadin for recent deep vein thrombosis who presents with significant vaginal bleeding x 1 day
Vital signs: BP 95/60, HR 120, RR 20, and afebrile.
General: Anxious and is uncomfortable.
Cardiovascular: Tachycardia, no murmurs.
Pulmonary: No tachypnea
Gynecologic: Bright red blood from the cervix with blood in the vault
LABS: Hgb: 4.5 g/dL , INR at 8.5
You make the decision to admit the patient to gynecology. In the ER you initiate a blood transfusion, hold warfarin, and administer reversal agents. Her nurse asks for the consent for transfusion of blood products.
transfusion consent
-Consent for all transfusion (unless emergency circumstances do not allow)
-Ex. for pRBCs:
-Patients with blood loss causing anemia often require transfusion. In general, this is a safe process that leads to improvement in your condition. However, there are risks associated with blood product transfusions. These can vary from simple issues that resolve without treatment, to more serious problems demanding quick and decisive interventions.
-Infectious: Hep B (1/300,000), Hep C (1/1.5 million), HIV (1 / 2 million units)
-Transfusion reactions:
-Febrile non-hemolytic reactions (1-3%) ( most common)
-Minor allergic reaction (1-3%)
-Acute hemolytic reactions – Life threatening – ABO incompatibility-> this means you gave the wrong blood type
-Anaphylactic 1/20,000
-TRALI 0.1%
-if reaction:
-STOP transfusion
-Change IV lines
-Call blood bank for guidance
-Send: T&C, DAT, CBC, CMP, haptoglobin, fibrinogen, LDH, PT/PTT, and all labels of products
