66 y/o m with fatigue and fever, with hx of Myelodysplastic syndrome
fever, tachycardia, petechiae, conjunctival pallor
elevated WBC, decreased Hgb, decreased PLT
Peripheral blood 25% blasts with large nuclei and visible nucleoli
Leukocytosis with ANC of 186 (neutropenic)
Dx: Acute Myeloid Leukemia with infection
Treatment for pt with AML and infection?
Bone marrow shows 55% blasts, start on chemotherapy with antibiotics for presumptive infection, transfusion support as needed
recheck blast count after two weeks
Pt with AML and infection, has recurrent fever after abx treatment. What do you do?
he's severely neutropenic from chemo, do a culture from central line. Make sure to check for fungal infection if gram stain is negative
treat bacteria/fungus and treat neutropenia with GM-CSF
What are the three phases of Chemo treatment?
Induction: initial chemo to induce a remission
Consolidation: intensify chemo to make sure no cancer cells are left in body
Maintenance: longer term therapy to maintain remission
Consider stem cell transplant for some cancers
What is the prognosis for our pt with AML?
age >60 and pre-existing MDS are concerning and there is a high rate of relapse
needs close monitoring
Next pt, 38y/o fm with complaints of weakness, bruising, menorrhagia
tachycardia, purpura, conjunctival pallor,
leukopenia, low Hgb, severe thrombocytopenia
How did we determine our 38y/o f pt has DIC?
fibrin clots forming/degrading leads to increased D-Dimer
What is underlying condition is causing the 38y/o f pt's DIC?
WBC isn't high, but circulating blasts show Auer rods
She is positive for t(15;17) which confirms Acute Promyelocytic Leukemia
How can Acute Promyelocytic Leukemia be treated?
PLT transfusion and factor replacement
ATO: arsenic trioxide
ATRA: all trans retinoic acid
68yo female with fatigue, weight loss, abdominal fullness
exam shows splenomegaly
low Hgb, low reticulocytes, normocytic RBCs
Leukocytosis of 87,400 with mature neutrophils, eosinophils and basophils
In the 68yo female with splenomegaly and severe leukocytosis, what does the bone marrow biopsy show? What does the blood smear show?
What translocation do you see?
What diagnosis does this lead to?
hypercellular bone marrow with mature granulocytes
macrophages with blue green cytoplasm (Pseudo-Gaucher cells)
t(9;22) (BCR-ABL TK)
Chromic Myelogenous Leukemia
How do we treat our pt with CML?
It is a TK inhibitor
Can monitor amount of BCR-ABL with cytogenics, FISH, or PCR to look at the molecular response to treatment
also follow the hematologic response to treatment as well
Why is it important to monitor CML closely?
It can progress to a more aggressive state
Two phases include:
Accelerated Phase: circulating blasts 10-19% with thrombocytopenia and basophilia
Blast Phase/crisis: blasts >20%
What about LAD makes you suspicious?
size greater than 4cm
(tender is often benign)
What are three ways that you can test LN when you find LAD?
Fine needle aspiration
What is a fine needle aspiration?
it is a liquid sample that can show metastatic cells or be used for flow cytometry to assess the clonal nature of the cells
It is only used for NHL
What is a needle biopsy used for?
it examines a solid core of tissue and can pull out metastatic cells or be used for flow cytometry
What is an excisional biopsy used for?
Best way to diagnose lymphoma and the ONLY way to diagnose HL via Reed-Sternberg cells
14y/o f pt with abdominal pain in RLQ. CT shows mass adjacent to appendix. Pt started on Abx for presumptive appendicitis. Repeat CT 2 days later shows increased mass size. Pathology shows Burkitt Lymphoma. What would further pathology reports show?
Is there any metastasis?
What is the treatment?
MYC translocation of t(8;14)
WORST lymphoma translocation
intracranial mass and bone marrow involvement (CNS and BM involvment more likely with Burkitt's than other NHL)
chemo with intrathecal admin.
In our pt with Burkitt's Lymphoma, urine output decreases after chemotherapy initiation. What does her labwork show and why is this happening?
How do you treat?
Increase in plasma K, serum creat. and BUN
this is indicative of Tumor Lysis Syndrome
Treat with aggressive hydration
(the tumor burden and high response to chemo increases risk for TLS)
22 y/o male with fatigue, chest pain after ETOH consumption, night sweats and fever, pruritis, weight loss, no LAD or splenomegaly. Because of these B symptoms you do what type of test?
What does the pathology show?
What does this inidicate?
How do you treat it?
Excision biopsy of chest mass seen on imaging
Path shows Reed-Sternberg cells
Indicates a HL
Treat with chemo and mayne some radiation
When it comes to HL, what are three common ways of presentation?
B-symptoms (can be in NHL)
Mass on chest x-ray
(may be limited to mediastinal mass that produces minimal or no local mass effect sx)
Pt is an older male with conjunctival pallor, petechiae, and axillary/cervical LAD.
What do labs show?
What is the diagnosis?
slightly elevated WBC, low Hgb, decreased PLT
Blood smear shows 90% small lymphocytes
Flow cytometry shows monoclonal B cells
In our pt with CLL/SLL, his wife mentions that he was diangosed with CLL many years ago and told to return for monitoring, but he did not do so. REcords show that 6yrs ago, he had CLL present without anemia or thrombocytopenia. Now that he has new cytoppenias, what does this mean for the progression?
There is now bone marrow involvement indicating acceleration of the dz.
This can become Richter's Transformation
13 y/o f with hx of menorrhagia, easy bruising, scrapes take a long time to stop bleeding, occasionally nose bleeds 1-2x per month. Dad with similar hx.
What do labs show?
What is the diagnosis?
How to treat?
low hgb, normal PLT
PTT prolonged, PT normal
vWF ag: decreased
Factor VIII: normal
Dx: vWF Disease Type I
Tx: Desmopressin to help release vWF
Bonus genetics: 1, 2a and 2b are AD
6 y/o m with oral bleeding after dental procedure, hx of of easy bruising from minor trauma and bleeding into joints
normal PT, prolonged PTT
Factor XIII: only 3% normal activity
Factor IX: 98% normal activity
geneitc test for Hemophilia A: positive
Recombinant Factor XIII
What can an abnormal aPTT alone indicate?
if bleeding: VIII, IX, XI defects
no bleeding: XII, prekalikrein, kininogen, lupus, anticoagulants
What does an abnormal PT alone indicate?
What does an abnormal PT and aPTT together indicate?
anticoagulant therapy, DIC, liver disease, K deficiency, massive transfusion
rarely factor X, V, II defects
32y/o female with recurrent vaginal bleeding 6wks post partum with epistaxis and fatigue. Family history of autoimmune disease.
What should we further test?
How do we treat?
ecchymosis, pallor, hypotension and tachycardia, vaginal bleeding
anemia with normal PT and prolonged PTT that was not corrected with mixing study (therefore it isn't a factor issue)
Test for antibodies. Negative Lupus/heparin, positive factor VIII inhibitor
fluids, factor VIII and factor VIIA bypass and immunosupression