Hem Onc Flashcards
Different types of white blood cells
Neutrophils - increased means infection - bands suggest bacterial infection
Lymphocytes: increased means viral infection
Eosinophils: parasites, allergic conditions
What diseases only have spherocytes on a peripheral blood smear?
Spherocytes are spheroid RBCs rather than bi-concave
Autoimmune hemolytic anemia - also has a positive direct Coombs test
—the spleen sees the spherocytes and identifies them as abnormal so it destroys them, therefore causing anemia
What are acanthocytes
RBCs that have a spiked cell membrane due to abnormal thorny projections.
“spur cell”
Usually caused by liver disease
What are schistocytes
RBC fragments as a result of mechanical destruction (fragmentation hemolysis) of a normal RBC.
Occurs when there is damage to a blood vessel and a clot begins to form
What are Howell-Jolly bodies
nuclear remnants in the cytoplasm
indicate splenic dysfunction
What do bands indicate?
These are immature white blood cells that are released when there is an infection
What might metamyelocytes suggest?
These are immature band cells
Indicate very immature WBC being sent out. Likely represents sepsis.
What do smudge cells indicate
remnants of cells that lack any identifiable cytoplasmic membrane or nuclear structure
Associated with chronic lymphocytic leukemia
What do blasts indicate if seen in the peripheral blood?
Usually indicates a leukemia or severe myelodysplastic syndrome
These are cancerous and should raise alarm
What are the three causes of anemia
-Problem with RBC production, usually in bone marrow
-Premature destruction of RBC (hemolysis) faster than new ones can be made
-Loss of blood
What are the three types of anemia
Microcytic: MCV <80
Normocytic: MCV 80-100
Macrocytic: MCV >100
Work up for microcytic anemia
Check iron levels
-If deficient (MOST LIKELY), work up for source of chronic blood loss
-if normal or elevated, consider anemia of chronic disease
Generally is caused by iron deficient anemia
Work up for normocytic anemia
Check a reticulocyte count (immature RBC)
-If low, anemia of chronic disease or bone marrow disease
-If elevated, blood loss or hemolysis
Work up for macrocytic anemia
Check B12/folate levels
-If low, vitamin B12 deficiency - Most likely - Pernicious anemia
-If normal, consider thyroid, ETOH, liver disease
Hemachromatosis: Causes, clinical presentation and treatment
Clinical syndrome of iron overload that is either caused by primary cause (gene mutation) or a secondary cause such as excessive blood transfusions or taking too many supplements.
Presents with interference of organ functions:
-Cirrhosis
-Bronzing of the skin
-CHF, arrythmias
-Endocrine problems
Treatment is phlebotomy to reduce ferritin
In primary gene mutation - can give Deferasirox (Exjade)
Lab findings in Thalassemia
Anemia with microcytosis and hypochromia
(low MCV and low MCHC)
Paroxysmal nocturnal hemoglobinuria - clinical presentation
-First morning urine is often dark or red (hgb)
-Prone to thrombosis especially in the skin (forms nodules), the hepatic and mesenteric veins
-Erectile dysfunction
-Esophageal spasm
Labs show thrombocytopenia, hemoglobinuria
What is autoimmune hemolytic anemia: Causes and presentation include labs
Increased RBC destruction
Intrinsic causes: spherocytes,
-hemoglobinopathies: thalessemia, sickle cell
Extrinsic - immune response, infx (malaria, babeiosis)
-All are caused by hyperbilirubinemia
—Could get GGT which will be normal because LFTs will all be elevated in hemolytic anemia and GGT only elevated in liver disease
-Positive Coombs test
-If you see shystocytes - autoimmune hemolytic anemia
Will present with rapid onset anemia, plus jaundice and splenomegaly
Treatment of autoimmune hemolytic anemia
Prednisone 1-2mg/kg/day
Plasmapheresis
Splenectomy
Transfuse as needed
Treatment of autoimmune hemolytic anemia
Prednisone 1-2mg/kg/day
Plasmapheresis
Splenectomy
Transfuse as needed
Polycythemia Vera - Cause and presentation
Mutation of the JAK2 gene results in the overproduction of all blood cells, causing an expanded blood volume and increased blood viscosity
Patients usually complain about pruritus, headache, dizziness, pulsatile tinnitus
Usually appear ruddy complexion and look swollen
Biggest complication is the blood viscosity and arterial thrombosis
Treatment of polycythemia vera
Regular phlebotomy to keep HCT <45
Hydroxyurea can be used as well if phlebotomy is not tolerate
Ruxolitinib is the only JAK2 inhibitor approved by the FDA
Disorders that cause thrombocytopenia
Immune thrombocytopenia purpura (ITP)
-Diagnosis of exclusion
-Sxs include spontaneous bruising, petechial rash, spontaneous bleeding from the nose, gums, vagina
-Platelet count will be very low
-Caused by decreased production due to destruction of the metakaryocytes
-Treatment can be supportive and it will resolve on its own or can give prednisone
Thrombotic Thrombocytopenic Purpura (TTP)
-Rare, hem emergency
-ITP plus hemolytic anemia
-blood clots form in small vessels throughout the body
-This causes low platelet count, low RBC count, and kidney, heart and brain dysfunction
-Presentation is fevers, AMS, thrombocytopenia, acute renal failure, hemolytic anemia
-Treatment is plasmapheresis and usually Rituximab
—Do not give platelets as this will only fuel the disease
Heparin-induced thrombocytopenia (HIT)
The immune system forms antibodies against heparin when it is bound to a protein called platelet factor 4
If you see platelet counts falling within 5 days of starting heparin, should stop heparin right away and start alternative anticoagulant
–bivalirudin, argatroban, fondaparinux
—This treats the clots that are formed because of the HIT
—Do not start Warfarin
Disseminated Intravascular Coagulation (DIC)
Clotting cascade is activated either locally or systematically
Condition is always secondary to something else
-Sepsis
-Retained products of conception
-Malignancy, often acute leukemia
-Starts with inappropriate activation of the clotting cascade and platelet activation
-As it progresses, clotting factors and platelets are exhausted and spontaneous bleeding begins
Treatment of DIC
Treat the trigger
Establish a baseline with lab parameters
-Platelet count
-PT, PTT
-D-Dimer
Transfuse blood products
Follow the lab parameters every 4 hours
If bleeding persists, consider heparin infusion if the platelets are >50 but contraindicated if the GI or CNS bleeding, placental abruption, need for operative management
Von Willebrand Disease: Cause and Presentation
The most common congenital disorder of coagulation
-Deficiency in the quality or quantity of von Willebrand factor - a protein required for platelet adhesion
Presentation ranges from mild bleeding that often goes undiagnosed because it is so small to severe bleeding with even minor trauma
Treatment of Von Willebrand Disease
For most patients it is DDAVP (Desmopressin)
If more severe bleeding, can give Factor VIII
Hemophilia - Causes and complications
X-linked recessive disorder (therefore only occurs in males)
Hemophilia A is a deficiency of Factor VIII
Hemophilia B is a deficiency of Factor IX
Diagnosis:
Generally done by genetic counseling and diagnosed during pregnancy
Complications:
Deep internal bleeding from minor trauma
Joint damage from hemarthrosis
Spontaneous intracranial hemorrhage
Treatment:
DDAVP for mild cases
Severe cases: regular administration of recombinant factors
Reversal of anticoagulation
Heparin and LMWH - Protamine
Fonaparinux - cannot be reversed with Protamine
Warfarin - Vit K, K centra
DOACs - short half life so usually can just stop it
–Pradaxa - Praxabind
–Xarelto and Eliquis - Andexxa
Difference between acute and chronic leukemias
Acute: Rapid increase in the number of immature blood cells
–Immediate treatment is needed because of the rapid progression and accumulation of blasts which can spill into blood stream and spread to other organs
—Usually easier to treat because the cells are so immature and differentiating so quickly that they are vulnerable
Chronic: Excessive buildup of relatively mature, but still abnormal WBC
–Usually seen in older patients
—Usually monitored for a bit to determine treatment course
Chronic Myeloid Leukemia (CML) - cause, presentation, treatment
-Caused by a chromosomal abnormality at the Philadelphia chromosome*** HALLMARK
-Early stages are asymptomatic and usually picked up incidentally on routine lab studies
-Can present in acute form - rapid progression, low survival rate - “blast crisis”
—spontaneous bleeding, fevers, petechia
In chronic form, presents with fatigue, bone pain, splenomegaly
Labs will show pronounced leukocytosis without obvious infection
–Mature myeloid cell types predominate
—Bone marrow biopsy shows the bcr/abl genotype
Treatment:
-Since often found in the elderly, treatment may be deferred
-If undertaken, usually treated with Tyrosine kinase inhibitors with good prognosis at 5 years
–Curative with bone marrow transplant
Myelodysplastic Syndrome
Acquired clonal disorder of the hemotopoietic stem cell
—These cells are disorder and they make clones of themselves
These cells stay in the marrow and do not leave
Caused by radiation and chemotherapy
Many patients had cancer before, were treated with chemotherapy, went into remission and then develop MDS because of the chemo.
—They then will progress to an Acute Myeloid Leukemia
Treatment is supportive and blood products
-Also hematopoietic growth factors
Prognosis is months to years
Acute Myeloid Leukemia - Presentation and lab findings
More common in adults, specifically males
Presentation is usually fatigue and spontaneous bleeding
Labs: Leukocytosis, severe anemia, thrombocytopenia, blasts appear on the diff
Malignant cell in AML is the myeloblast
