Term
| What are some other myeloproliferative disorders other than myelofibrisis? |
|
Definition
CML
PCV
Essential thrombocytosis |
|
|
Term
| What labs indicate CML vs. other myeloproliferative disroders? |
|
Definition
WBC: Marked increase
Hct: Normal
Plt: Normal or increased
RBC: Normal |
|
|
Term
| What labs indicate Myelofibrisis vs. other myeloproliferative disroders? |
|
Definition
WBC: Normal, increased or decreased
Hct: Normal or decreased
Plt: Normal, increased or decreased
RBC: Tear drop |
|
|
Term
| What labs indicate PCV vs. other myeloproliferative disroders? |
|
Definition
WBC: Normal or increased
Hct: Increased
Plt: Normal or increased
RBC: Normal |
|
|
Term
| What labs indicate Essential thrombocytosis vs. other myeloproliferative disroders? |
|
Definition
WBC: Normal or increased
Hct: Normal
Plt: Increase
RBC: Normal |
|
|
Term
| How do you treat hemorragic anemia? |
|
Definition
Fluid resuscitation
RBC ransfusion
Look for cause of bleeding |
|
|
Term
| How do you treat hereditary spherocytosis? |
|
Definition
Splenectomy
Vaccine against encapsulated organisms (N. meningitidis, S. pneumonia, H. influenzae type b) |
|
|
Term
| How do you treat Autoimmune hemolytic anemia? |
|
Definition
Steroids
Immunosuppresive agents
IVIG
Consider splenectomy |
|
|
Term
An 8yo boy eats at a fast food burger which was found to be contaminated with bacteria. Two days later, he develops bloody diarrhea. On HD4, he has dark urine. Hbg is 8.5, plt 41,000, creatinine lvl is 5.6, schistocytes seen.
What is the Dx and what is next step? |
|
Definition
HUS (hemolytic uremic syndrome)
Hemodialysis |
|
|
Term
| What is the mneumonic for TTP - a medical emergency? |
|
Definition
Run FAST!
Renal Failure
Fever
Anemia (microangiopathic-hemolytic)
Seizure (CNS dysfunction)
Thrombocytopenia |
|
|
Term
| What are some causes of DIC? |
|
Definition
Sepsis
Shock
Malignancy
Obstetric complications
Trauma |
|
|
Term
| What are the symptoms of HUS? |
|
Definition
Hemolytic anemia
Thrombocytopenia
ARF |
|
|
Term
| What is the most common cause of HUS? |
|
Definition
Viral illness
E coli O157:H7 (more common in >10 yrs) |
|
|
Term
|
Definition
Fever
Fluctuating neurologuc signs
Hemolytic anemia
Thrombocytopenia
ARF |
|
|
Term
| What are some causes of TTP? |
|
Definition
|
|
Term
|
Definition
Treat underlying condition
Transfuse platelets
Give protein C concentrate of cryoprecipitate (replaces fibrinogen)
FFP (to replace coagulation factors) |
|
|
Term
| What do the labs look like for TTP or HUS? |
|
Definition
Plt: Markedly decreased
PT/PTT: Normal
D-Dimer: Normal
Other: ARF, CNS dysfunction |
|
|
Term
| What do labs look like for DIC? |
|
Definition
Plt: Decreased, but not as much as TTP
PT/PTT: Increased
D-Dimer: Markedly increased
Fibrin split products: Increased
Fibrinogen: Decreased
D-Dimer: Increased |
|
|
Term
| What do the labs look like for Mechanical valve Hemolysis? |
|
Definition
Plt: Normal
PT/PTT: Normal
D-Dimer: Normal
Heart murmer |
|
|
Term
| What do labs look like for Severe vasculitis, severe hypertension, or HELLP? |
|
Definition
Plt: Decreased
PT/PTT: Normal
D-Dimer: Normal
Liver enzymes: Elevated in HELLP |
|
|
Term
|
Definition
| Dialysis when ARF is present |
|
|
Term
|
Definition
Plasmapheresis
FFP if urgent
DO NOT GIVE PLATELETS! (May exacerbate TTP) |
|
|
Term
| Five yo girl with Sickle cell dz is in class with a child who recent came down with a facial rash. The girl's mother calls you for advice. What complication is a concern? |
|
Definition
| Aplastic crisis due to parvovirus B19 (causes fifth's disease). |
|
|
Term
| What is seen with sickle cell presentation? |
|
Definition
Predominatly in African Americans with + family history.
Gallstones (occurs with chronic hemolysis)
Poorly healing ulcers
Jaundice
Splenomegaly in kids
CHF |
|
|
Term
| How do you diagnose sickle cell anemia? |
|
Definition
Blood smear shows sickled cells
Howell-Jolly bodies
Evidence of hemolysis
Hemoglobin electrophoresis is definitive diagnostic test. |
|
|
Term
| What are the characteristics of Pain crisis in Sickle cell anemia? |
|
Definition
| Pain in the back, limbs, abdomen and ribs: precipitated by dehydration, acidosis, infection, fever or hypoxia. |
|
|
Term
| What is the treatment of pain crisis in sickle cell anemia? |
|
Definition
| Hydration, analgesia, supplemental O2 |
|
|
Term
| How is aplastic crisis triggered in sickle cell and how do you treat? |
|
Definition
Presents as sudden decrease in Hb and reticulocyte count due to parvovirus B19.
Support with transfusion. |
|
|
Term
| What is Acute chest syndrome in sickle cell? |
|
Definition
Infection, infarction and fat PE
Fever, chest pain, cough, wheezing, tachypnea, new pulmonary infiltrate on CXR
Give O2, analgesia, transfusion, and antibiotics( 2nd or 3rd gen cephalosporin with erythromycin |
|
|
Term
| How does sickle cell effect the lungs? |
|
Definition
| Pulmonary infarcts can cause pulmonary hypertension |
|
|
Term
| How does sickle cell effect the heart? |
|
Definition
| Sickle cell cardiomyopathy may lead to heart failure |
|
|
Term
| How does sickle cell effect the GI tract? |
|
Definition
| Cholecystitis, splenic infarcts |
|
|
Term
| How does sickle cell effect the kidneys? |
|
Definition
| infarcts, papillary necrosis, ARF |
|
|
Term
| How does sickle cell effect the genitals? |
|
Definition
| Priapism or impotence in males |
|
|
Term
| How do infections cause a problem in sickle cell? |
|
Definition
| Absence of functional spleen predisposes patients to encapsulatd organisms, including S. pneumoniae, H influenzae, N meningitidis, and G- bacterial infections |
|
|
Term
| How does sickle cell effect the bones? |
|
Definition
Avascular necrosis
Salmonella osteomyelitis |
|
|
Term
| How does sickle cell effect the CNS? |
|
Definition
|
|
Term
| How does sickle cell effect pregnancy? |
|
Definition
|
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