Splenic Disorders & Leukemia Lymphoma Cases Flashcards
What is Red pulp?
- Sinusoids network filled wit hblood that filters abnormal RBC and platelets out
What is white pulp?
- Marginal zone:
- area where macrophages responsible for clearing out debris and encapsulated organisms are found
- PALS: T cells
- Primary follicle: B cells antibody production
Why is splenomegaly a problem?
- Rupture in acute splenomegarly
- Staging of hematologic malignancies
- Portal hypertension
- Cytopenias in certain cases (leads to hypersplenism)
Splenomegaly vs Hypersplenism
Splenomegaly:
- enlarged spleen, can be an active or passive process
Hypersplenism:
- Pathologic condition where blood cells are removed by the spleen leading to anemia, leukopenia, or thrombocytopenia on a CBC
Examples of hypersplenism?
- Sickle Cell Anemia:
- Life threatening condition caused by pooling of blood in the spleen
- HS:
- splenic sequestration and luysis due to cellular shape and can worsen anemia
- Idiopathic thrombocytopenic purpura:
- Acquired autoantibodies against platelets
- Cases refractory to pharmaceutical therapy or cases with life threatening thrombocytopenia benefit from splenectomy
What is Castell’s point?
Palpate at the anterior axillary line under ribcage and have patient breathe in, if they have splenomegaly you will feel the spleen
- If pretest suspicion is high you can use PE
- If pretest suspicion is low use imaging
Common causes of splenic rupture?
- Trauma is most common
- Splenomegarly due to mono, CLL or HCL
- Also caused by surgical trauma
What is Kehr’s sign
- Irritation of phrenic bnerve by sub diaphragmatic bleeding
- Left shoulder pain
- Elicited in recumbent position with legs raised
When can a patient with mono return to non contact sports? contact sports?
- non: 3 weeks from onset of sx
- contact: 4 weeks from onset of sx
What is OPSI?
- Overwhelming post splenectomy infection
- Occurs bc spleen is removed and can’t fight encapsulated organisms anymore
- no spleen=no reticularendothelial system
- Starts vague illness progressing to sepsis in 2-3 days
- Highest risk is 3 yrs after splenectomy
What vaccines do patients who had a splenectomy need?
- S. pneumoniae
- N. meningitidis
- H. influenzae B
66 yo man with fatigue and fever complaining of weakness and bruising. He has petichiae on feet and arms and conjunctival pallor.
- Hgb: 7.6
- Hct: 22
- WBC: 18.6
- Plt: 60
Peripheral smear shows 25% blasts, they are large cells with large nuclei and visible nucleoli. The patient previously had MDS. What are you now suspecting? How does this explain his symptoms?
AML arising from MDS
- Pallor from anemia
- Petechiae and brusing from low platelets
- Fever from non functional WBC’s
What tx do you do for neutropenic fever?
- Empiric antibiotics
- Empiric abx are targeted at gram nexative organisms first (psuedomonas)
- Cefepime, piperacillin-tazobactam, meropenem, ceftazidine
- Fungal coverage added after 48n hrs if fever persists
What should you do if your patient has a neutropenic fever after prior abx tx during induction chemo, but cultures come up negative for bacteria?
- Silver stain for fungus from the central line
- Treat with amphotericin B/voriconazole and replace the line
- Treat with GM-CSF to get the neutrophil count back up
What are the three stages of chemotherapy?
- Induction: initial chemo to induce a remission
- Consolidation: intensifying chemo to make sure no cancer cells survived
- Maintenance: for some cancers long term therapy is needed to maintain remission and prevent relapse
66 yo male diagnosed with AML with pre existing MDS, what is his prognosis after chemo?
- initial response is good, but greater than 60 yo and prior MDS leads to higher rate of relapse and tx complications
38 yo mother of 15 yo daughter and 17 yo son comes into ER complaining of weakness, bruising, heavy period. HR is 125 bpm, she has purpura on torso and extremeties, and conjunctival pallor. CBC and labs show:
- PT: 23 sec (high)
- PTT: 87 sec (high)
- Fibrinogen 45 (low)
- D-dimer: 800 (high, normal <250)
- Hbg: 9.3
- Hct: 28
- Plt: 20
- WBC: 3.6
Smear and chromosomal studies show circulating blasts with auer rods and a PML/RARa translocation. What is the diagnosis? What is the patient at high risk for?
- APL (15:17 translocation)
- DIC
How do you treat someone with APL?
ATRA and ATO
68 yo woman with fatigue, 10 lb weight loss in 2 months and abdominal fullness. PE shows mild splenomegaly. CBC shows:
- 11.5 hgb (low)
- 35 hct (low)
- 87.4 WBC (high)
- 289 plt (normal)
- Retic normal
- Normocytic
- Low LAP
What is LAP indicating?
Bone marrow aspirate shows hypercellular marrow consiting of mature granulocytes and psuedo gaucher cells. What are psuedo gaucher cells and what condition is indicated? What would the translocation be?
- Positive LAP staining indicates reactive conditions and absent staining indicates abmormal myelocyte populations
- Macrophages that clean up cell debris when ther is a high rate of turnover in the marrow
- CML
- t(9:22) constitutively active tyrosine kinase, treat with gleevec
What is the most sensitive way to test for BCR-ABL?
PCR
What is the best way to diagnose lymphoma and ONLY way to diagnose Hodsgkin lymphoma?
Excisional biopsy
What is fine needle aspiration used for?
Only Non Hodgkin lymphoma
What is one of the worst lymphoma translocations?
8:14 IgH/MYC seen in Burkitts
What is seen in tumor lysis syndrome? Why does it occur? How do you treat it?
- Hyperkalemia
- Hyperuricemia
- Hyperphosphatemia
- Hypocalcemia
Fast growing tumors have high probability of TLS
Tx with allopurinol, a Xanthine oxidase inhibitor that prvents uric acid formation, also treat the electrolyte imbalances
What lymphoma is more likely to have CNS and bone marrow involement than other NHL types?
- Burkitt’s
- Due to the CNS risk, intrathecal chemo is given
What are the three ways HL typically presents?
- Lymphadenopathy
- Constitutional B symptoms
- Accidently found on a Cxr
65 yo man with complaints of fatigue, PE shows conjunctival pallow, petechiae and axillary and cervical LAD. CBC shows:
- 14.6 WBC high
- Hbg low
- Platelets low
- Peripheral smear shows 90% small lymphocytes
- Flow cytometry shows mmonoclonal B cells
Patient had been diagnosed with CLL/SLL 6 years ago, but did not have the anemia or thrombocytopenia.
What is happening?
- Richter transformation, this is when CLL/SLL transforms to a more deadly aggressive DLBCL
- Only occurs in 3-5% of patients with CLL
- need to start tx asap as 100% mortality in weeks if not tx, but with tx survival is poor at roughly one year
- Tx is chemo and Rituximab
What is Rituximab?
Monoclonal ab against CD20 cell receptors
