Singh's Case Studies Flashcards
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
prolonged PTT/PT
low fibrinogen
D-dimer elevated
Dx: DIC
How did we determine our 38y/o f pt has DIC?
Low PLT
Increased PT/PTT
decreased fibrinogen
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
Chemotherapy:
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
normal PLT
low LAP
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?
Imatinib
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?
Subclavian/Supraclavicular nodes
fixed/hard nodes
size greater than 4cm
(tender is often benign)
What are three ways that you can test LN when you find LAD?
Fine needle aspiration
Needle Biopsy
Excision Biopsy
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?
LAD
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
Dx: CLL/SLL
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?
Bonus genetics?
low hgb, normal PLT
PTT prolonged, PT normal
vWF ag: decreased
vWf: 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
Labs show?
Diagnosis?
Genetics?
Treatment?
normal PT, prolonged PTT
Factor XIII: only 3% normal activity
Factor IX: 98% normal activity
geneitc test for Hemophilia A: positive
X-linked recessive
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?
VII defect
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.
Exam shows:
Labs show:
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
What happens if a mixing study of PT/PTT corrects?
If it doesn’t correct?
this means that there is a factor deficiency
means there is an inhibitor antibody present in the blood
Acquired bleeding disorders can be of two varities:
Primary hemostatic disorders
Secondary hemostatic disorders
Primary:
drug induced platelet destruciton
aplastic anemia
ITP
mechanical PLT destruction (hypersplenism)
Secondary:
autoimmune inhibitors (VIII and V)
antiphospholipid antibodies
drug induced factor inhibition (heparin. warfarin)
consumption of factors
What is acquired hemophilia A
Factor VIII inhibitor
seen in odler adults or post-partum
can be associated with other autoimmune diseases
may be seen in malginancy
Can occur in congenital hemophilia A as well
What makes you suspect a DVT/PE?
Wells, Geneva criteria
- hx of previous PE/DVT
- cancer pt
- immobilization?
- hemoptysis
- tachycardia
- over 65
How do you diagnose a thromboebolism?
What is a benefit of the D dimer?
D dimer
US or CT angiogram or VQ scan
high negative predictive value: if negative, no thromboembolitic event
36 y/o female with abomdinal pain and vomiting. hx of SAB 12 years ago and on OCP now. Mother died of stroke at age 45.
Exam:
Dx:
What is the underlying cause for this diagnosis?
occult blood in stool and ileus on CT scan
WBC elevated with left shift
Bowel infaction due to mesenteric vein thrombosis?
Protein C def.
Why did we diagnose our ileus pt with protein C deficiency?
How to treat?
current mesenteric vein thrombosis with hx of miscarriage and family history of you stroke
being on OCP can increase risk for clot
stop OCP, start on anticoagulation
White thrombus is characterized how?
arterial
PLT rich
areas of shear stress
ATHEROSCLEROSIS
coronary/cerebral arteriers
Red thrombus is characterized how?
venous
red cell rich
STASIS
lower extremities
In the pt with protein C def., how did her mother’s stroke occur?
likely started as a DVT that ended up as a paradoxical embolism passing through a patent foramen ovale and getting into arterial circulation, going to brain and causing damage
found inf 55-60% of patients who have strokes under age 55
68 y/o male with 3 months of fatigue and dyspnea on exertion, brittle nails, weight loss, pica (drinking paint)
Exam?
Labs?
Peripheral smear?
Diagnosis?
pale, conjunctival pallor, stomatitis, stable vitals, onychoscizia.
anemia with low MCV (microcytic) and increased RDW
ferritin: low
transferrin: high
transferring % sat: low
smear shows anisocytosis (increased RDW)
dx: iron deficient anemia
Why is RDW helpful in anemia?
RDW is the earliest and most sensitive index that will change in iron deficient anemia
What are some lab indications that iron is low?
What are some indications that the body is waiting for iron (ie. compensating) ?
low serum iron
low to normal serum ferrtin
low transferrin/TIBC saturation (stores/capacity)
increased TIBC, increased TFR
In the elderly man with IDA can we just treat and send home?
No!
Look at occult blood in stool for GI bleed
Guess what, positive. Do scope and figure out what the underlying issue is.
35 yo m with coffe grounds emesis x3 and 3 month hx of epigastric pain relieved by OTC antacids. hx of smoker and regular NSAID use. Begins to have chest pain.
Exam:
Labs:
EKG:
distended nontender abd, hypotenive and tachycardic
RRR with SEJ
cool extremities and weak pulse
Hgb low, normal reticulocytes, AB neg blood type
EKG: STEMI
In our hypovolemic pt who just had a heart attack, we give a rapid transfusion of 1L NS and two units of blood. Repeat H/H shows?
What does surgery show?
Hgb increased by 1. Should increase by 1 for every one unit given. Thus he’s actively bleeding, so we need to figure out where from
Large ulcer that is nearly perforated; treated and bleeding controlled
Blood group O
Blood that it can receive?
Plasma that it can receive?
Blood: Group O
Plasma: O, A, B, AB
Group A blood
Can receive?
Plasma it can receive?
blood: A and O
PLasma: A and AB
Group B blood
can receive which blood?
can receive which plasma?
blood: B and O
Plasma: B and AB
Group AB blood
Can receive which blood?
can receive which plasma?
blood: O, A, B, AB
plasma: AB
4 yo AA m with right leg pain after increasing elevation flying from nebraska to denver. Dyspnea on exertion. Fhx of some type of anemia.
Exam:
Labs:
Rad:
Smear:
Dx:
febrile, tachy, O2 sat at 91%,
Labs: Hgb low, MCV normal, fullness of LUQ
Femoral head fracture (pathologic)
smear shows cell sickling and elevated HgbS
Sickle Cell Anemia
What are the genetics of Sickle cell Anemia?
AR causing predominance of HgS caused by glutamate to valine mutation
high in AA populations
What are some effects of SCA?
Stroke
Acute chest syndrome (vasoocclusion in lungs leading to lower oxygenation)
renal issues with hematuria, even in SC trait
acute bone necrosis
autosplenectomy
What are some treatments for SCA?
oxygen, breathing exercises,NSAIDs and narcotics, avoid high altitudes and strenuous acivity
keep HbS low with Hydroxurea
consider stem cell transplant
25 yo f with hx of fatigue, weakness, dizziness, petechiae, pallor and gingival bleeding. She was recently treated with Bactrim for a UTI.
Labs show:
What do these labs indicate?
What is the diagnosis?
decreased WBC, decreased Hgb, decreased PLT
reticulocyte low
MCV high
RDW normal
either chemo/antivirals, ETOH or aplastic anemia
Aplastic anemia due to recent bactrim use
Treatment for aplastic anemia
trasfusion, growth factor support
possible stem cell transplant
Should you always try to palpate someone’s spleen?
probably not, unless you have a high index of suspicion for spleen related pathology
What are large spleens typically indicative of?
hematologic malingnancy (useful in staging)
liver disease with portal hypertension
large spleen itself may be worsening a disease process (causing cytopenias)
Hypersplenia is a type of splenomegaly with what features?
blood cells are being removed from body by the spleen
typically contributes to disease process
What three diseases can cause the spleen to capture or break cells?
sickle cell anemia
heridtary spherocytosis
ITP
SCA and the spleen
splenic sequestration of red cells can cause crisis
splenectomy can reduce need for transfusions
too many crises and you can get an autosplenectomy
HS and the spleen
splenic sequestration and lysis can worsen anemia
splenectomy improves anemia
ITP and the spleen
cases refractory to pharmaceutical therapy or cases with life threatening thrombocytopenia may benefit from splenectomy
Ab coated PLT in spleen are destroyed via RES
If you see a giant spleen on pathology, what is a likely underlying cause?
If you see nodules in the spleen, what is most likely?
Hairy cell leukemia, CLL/SS
HL, follicular lymphoma
While trauma is the most comon cause of splenic rupture, what are other causes?
splenomegaly from mono, CLL, hairy cell leukemia, surgical trauma
How to diagnose splenic rupture
think about it (make sure to check for it)
LUQ pain
Kehr’s sign (diaphragmatic irritation when lying down, radiates to shoulder)
What is overwhelming post-splenectomy infection
(when the surgeon says, “OPSI”)
bacteria that are resistant to opsonization are dealth with by the RES of the spleen
no spleen, no RES
starts with vague illness and progresses to fulminant sepsis in 2-3 days
highest risk 3yrs after splenectomy
make sure to vaccinate
after splenectomy, what vaccines do you need?
Strep pneumoniae
n. menigiditis
H. influenzae B
What on blood smear is seen after splenectomy/hyposplenia?
Howell-Jolly bodies (little dot in RBC)
