Flashcards in MTB Heam/Oncology Deck (72):
All ER or PR positive patients should receive
tamoxifen, raloxifene, or one of the aromatase inhibitors
All breast cancers should be tested
for Her2/neu. This is an abnormal estrogen receptor.
Hormonal Manipulation in Prostate Cancer
Flutamide, GNRH agonists, ketoconazole, and orchiectomy help control the size and progression of metastases once they have occurred.
Small cell cancer is considered unresectable in 95% of cases because
it is meta- static or spread outside one lung.
Diagnostic Testing of testicular cancer
Remove the whole testicle with inguinal orchiectomy. Do not cut the scrotum, which can spread the disease. Needle biopsy of the testicle is always a wrong answer.
Alpha fetoprotein is secreted only by nonseminomatous cancers. HCG is up in all ofthem.
The management ofadvanced cervical cancer is clear:
Perform a hysterectomy.
Prevention of Cervical Cancer
o Human papillomavirus (HPV) vaccine is given to all women between ages 11 and 26.
o Pap smear is performed starting at age 21. Repeat the test every 3 years until the age of 65. Of women with fatal cervical cancer, 85% have never had a Pap smear. Pap and HPV testing increase the interval to 5 years.
Causes of low MCV:
• Iron deficiency
• Sideroblastic anemia
• Anemia of chronic disease
Microcytic anemias generally have a low reticulocyte count.
Causes of high MCV:
• B12 a nd folate deficiency
• Sideroblastic anemia
• Antimetabolite medications such as azathioprine, 6-mercaptopurine, or
• Liver disease or hypothyroidism
• Medications such as zidovudine or phenytoin • Myelodysplastic syndrome (MDS)
Symptomatic from anemia means:
• Shortness of breath
• Lightheaded, confused, and sometimes syncope • Hypotension and tachycardia
• Chest pain
Vitamin B12 deficiency is caused by:
• Pernicious anemia
• Pancreatic insufficiency
• Dietary deficiency(vegan/strictvegetarian)
• Crohn disease, celiac, tropical sprue, radiation, or any disease damaging the terminal ileum
• Blind loop syndrome (gastrectomyorgastricbypassforweightloss)
• Diphyllobothrium latum,HIV
Folate deficiency is caused by:
• Dietary deficiency (goat's milk has no folate and provides only limited iron and B12)
• Psoriasis and skin loss or turnover
• Drugs: phenytoin, sulfa
Sickle cell is a
chronic, usually well-compensated hemolytic anemia with a reticulocyte count that is always high.
Other Common Manifestations of Sickle Cell Disease
• Increasedinfectionfromautosplenectomy,particularlyencapsulated organisms
• Enlarged heart with hyperdynamic features and a systolic murmur
• Lower extremity skin ulcers
• Avascular necrosis ofthe femoral head
Which of the following can be found on smear in sickle cell disease?
a. Basophilic stippling
b. Howell-Jolly bodies
c. Bite cells
"What Is the Most Likely Diagnosis?"
• Recurrent episodes of hemolysis
• Intermittent jaundice
• Splen omegaly
• Family history of anemia or hemolysis
• Bilirubin gallstones
Diagnostic Tests autoimmune hemolysis
The most accurate diagnostic test is the Coombs test, which detects IgG antibody on the surface of the red cells.
Cold agglutinins are
IgM antibodies against the red cell developing in association with Epstein-Barr virus, Waldenstrom macroglobulinemia, or Mycoplasma pneumoniae.
cryoglobulins are associated with:
• Hepatitis C
• Joint pain
Glucose 6 phosphate dehydrogenase (G6PD) deficiency is
an X-linked recessive disorder leading to an inability to generate glutathione reductase and protect the red cells from oxidant stress. The most common oxidant stress is infection.
The best initial test for G6PD
is for Heinz bodies and bite cells.
Paroxysmal nocturnal hemoglobinuria (PNH) is
a clonal stem cell defect with increased sensitivity of red cells to complement in acidosis.
• Episodic dark urine with the first urination of the day from hemoglobin
• Pancytopenia andiron deficiency anemia
Aplastic anemia is
pancytopenia of unclear etiology. Any infection or cancer can invade the bone marrow, causing decreased production or hypoplasia.
Polycythemia vera (p. vera) is
the unregulated overproduction of all3 cell lines, but red cell overproduction is the most prominent. There is a mutation in the JAK2 protein which regulates marrow production. The red cells grow wildly despite a low erythropoietin level.
Patients present with symptoms of hyperviscosity from the increased red blood cell mass such as:
• Headache, blurred vision, and tinnitus
• Bleeding from engorged blood vessels
• Thrombosis from hyperviscosity
Pruritus often follows warm showers because of histamine release from increased numbers of basophils.
a markedly elevated platelet count above one million leading to both thrombosis and bleeding.
painful, red hands in ET. Treat with aspirin.
Myelofibrosis is a
disease of older persons with a pancytopenia associated with a bone marrow showing marked fibrosis.
Look for teardrop-shaped cells and nucleated red blood cells on blood smear.
Patients present with signs of pancytopenia (fatigue, infection, bleeding) even though the white blood cell count is normal or increased in many patients. Despite an increase in white cell count, infection is a common presenta- tion because leukemic cells (blasts) do not function normally in controlling infection.
The most frequently tested type of acute leukemia is
M3 or acute promyelocytic leukemia.
This is because promyelocytic leukemia is associated with dissemi- nated intravascular coagulation (DIC).
M3 is associated with
translocation between chromosomes 15 and 17.
The best initial test for Acute leukemia is a
blood smear showing blasts.
acute myelocytic leukemia (AML) is characterised by
Auer rods are
eosinophilic inclusions associated
with AML. M3 or acute promyelocytic leukemia is most commonly associated with Auer rods.
• M3 (promyelocytic leukemia) gives
• Add xxx to M3.
• Auer rods =xxx.
• Add xxx to ALM
• M3 (promyelocytic leukemia) gives DIC.
• Add ATRA to M3.
• Auer rods =AML.
• Add intrathecal methotrexate to ALL
"What Is the Most Likely Diagnosis?"
Look for a patient with
a persistently high WBC count that is all neutrophils.
• Pruritus is common after hot baths/showers from histamine release from basophils.
• presents with early satiety, abdominal fullness, and left upper quadrant pain.
• Chronic myelogenous leukemia (CML) can present with vague symptoms of fatigue, night sweats, and fever from hypermetabolic syndrome.
• CML can present with high WBC on routine exam.
Tyrosine kinase inhibitors such as imatinib
Only a BMT can cure CML
Leukocyte alkaline phosphatase score (LAP) is
low in CML but high in reac- tive leukocytosis.
CML has the greatest likelihood of all myeloproliferative disorders to transform Into
acute leukemia (blast crisis). If CML is untreated, this will happen in 20% of patients a year.
In acute leukostasis reaction, it is more important to
remove the excessive white cells from the blood than to establish a specific diagnosis.
Myelodysplastic leukemia is a
preleukemic disorder presenting in older patients (over 60) with a pancytopenia despite a hypercellular bone marrow. Most patients never develop acute myelogenous leukemia because complications of infection and bleeding lead to death before leukemia occurs.
Pelger·Huet cells are found in
Chronic lymphocytic leukemia (CLL) is a
clonal proliferation of normal, mature-appearing B lymphocytes that function abnormally. CLL occurs over the age of SO in 90% of those affected. Many are asymptomatic at presentation with only a markedly elevated white cell count.
The most common symptom of CLL is fatigue. Other symptoms include:
• Lymphadenopathy (80%)
the conversion of CLL into high-grade lymphoma, happens in 5% of patients.
CLL Diagnostic Tests
The WBC count is usually at least above 20,000/flL with 80% to 98% lympho- cytes. Half of patients are hypogammaglobulinemic.
Hairy cell leukemia (HCL) presents in middle-aged men with:
• Massive splenomegaly
• Inaspirable "dry" tap despite hypercellularity of the marrow
In hairy cell leukemia, B-cells with
filamentous projections are seen on smear.
Non-Hodgkin lymphoma (NHL) is a
proliferation of lymphocytes in the lymph nodes and spleen. NHL is most often widespread at presentation and can affect any lymph node or organ that h as lymphoid tissue. NHL and CLL are extremely similar, but NHL is a solid mass and CLL is "liquid" or circulating.
o Painless lymphadenopathy
o May involve pelvic, retroperitoneal, or mesenteric structures o Nodes not warm, red, or tender
o "B" symptoms: fever, weight loss, drenching night sweats
NHL Diagnostic Tests
The best initial test is an excisional biopsy. The CBC is normal in most cases. High LDH levels correlate with worse severity.
The most common wrong answer is to do a needle aspiration of the lymph node. Aspiration is not enough because the individual lymphocytes appear normal.
Local disease (stage Ia and Ila): local radiation and small dose/course of chemotherapy
Advanced disease (stage III and IV, any "B" symptoms): combination chemotherapy with CHOP and rituximab, an antibody against CD20
C = cyclophosphamide
H = adriamycin (doxorubicin or "hydroxydaunorubicin")
0 = vincristine (oncovin)
P = prednisone
The definition, presentation, diagnostic tests, "B" symptoms, and staging of Hodgkin disease (HD) are the same as NHL. HD has Reed-Sternberg cells on pathology.
Hodgkin disease treatment
Stage Ia and Ila: local radiation with a small course of chemotherapy
Stage III and IV or anyone with "B" symptoms: ABVD
A = adriamycin (doxorubicin)
B = bleomycin
V = vinblastine D = dacarbazine
Adriamycin (or doxorubicin) is
Myeloma is an
abnormal proliferation ofplasma cells. These plasma cells are unregulated in their production of useless immunoglobulin that is usually IgG or IgA. IgM is a separate disease called Waldenstrom macroglobulinemia. These immunoglobulins do not fight infection but clog up the kidney.
"What Is the Most Likely Diagnosis?"
The most common presentation of myeloma is
bone pain from pathologic frac- tures. A pathologic fracture means that the bone breaks under what would be considered normal use. This is from osteoclast activating factor (OAF), which attacks the bone, causing lytic lesions. OAF is also the reason for hypercal- cemia. Infection is common because the abnormal plasma cells do not make immunoglobulins that are effective against infections.
the most common causes of death in myeloma.
Renal failure and infection
Multiple myeloma Diagnostic Tests
The first test done is usually an x-ray ofthe affected bone that will show lytic ("punched out") lesions.
Serum protein electrophoresis (SPEP) shows an IgG (60%) or IgA (25%) spike of a single type or "clone." This one clone is called a Monoclonal or "M" spike. Fifteen percent have light chains or Bence-Janes protein only.
The first step in the evaluation ofbleeding is determining ifthe bleeding seems related to
platelets or clotting factors.
Immune Thrombocytopenic Purpura
"What Is the Most Likely Diagnosis?"
• Isolated thrombocytopenia (normal hematocrit, normal WBC count)
Before splenectomy, give vaccination to:
• Neisseria menlngitidis
• Haemophllus /nfluenzae
Von Willebrand Disease (VWD) Definition
VWD is the most common inherited bleeding disorder with a decrease in the level or functioning of von Willebrand factor (VWF). It is autosomal dominant.
VWD "What Is the Most Likely Diagnosis?"
Look for bleeding related to platelets (epistaxis, gingival, gums) with a normal platelet count. VWD is markedly worsened after the use of aspirin. The aPTT may be elevated in half of patients.
Look for delayed joint or muscle bleeding in a male child, since the condition is X-linked recessive. Bleeding is delayed because the primary hemostatic plug is with platelets. The prothrombin time (PT) is normal and the aPTT is pro- longed. Mixing studies with normal plasma will correct the aPTT to normal. The most accurate test is a specific assay for factor VIII or IX. Treat mild cases with DDAVP. Severe bleeding with very low levels of factor VIII or IX is treated with replacement of the specific factor.
Factor XI Deficiency
Most of the time, there is no increase in bleeding with factor XI deficiency. With trauma or surgery, there is increased bleeding. Look for a normal PT with a prolonged aPTT. Mixing study will correct the aPTT to normal, as occurs whenever there is a deficiency of clotting factors. Use fresh frozen plasma to stop the bleeding.
Factor XII Deficiency
These patients have an elevated aPTT but there is no bleeding. There is no therapy needed.
Disseminated Intravascular Coagulation
Disseminated intravascular coagulation (DIC) does not occur in otherwise healthy people. Look for a definite risk such as:
• Abruptio placentae or amniotic fluid embolus
• Traum resulting in tissue factor release
There is bleeding related to both clotting factor deficiency as well as thrombocytopenia.
Diagnostic Tests for DIC
• Elevation in both the PT and aPTT
• Low platelet count
• Elevated d-dimer and fibrin split products
• Decreased fibrinogen level (ithasbeenconsumed)
The most common cause is factor V Leiden mutation. There is no difference in the intensity of anticoagulation. Use warfarin to an INR of2 to 3 for 6 months.
The 2 main syndromes are the lupus anticoagulant and the anticardiolipin antibody. Both cause thrombosis. Anticardiolipin antibodies are associated with multiple spontaneous abortions. The antiphospholipid (APL) syndromes are the only cause of thrombophilia with an abnormality in the aPTT.
ALS The best initial test is
the mixing study. Because it is a circulating inhibitor, the aPTT will remain elevated even after the mix. The most accurate test for the lupus anticoagulant is the Russell viper venom test.