Haemat SAQ

Question 1

54/M GPH, incidentally found 6cm splenomegaly during health check. Asymptomatic, with no hepatomegaly or lymphadenopathy. He is slightly pale. CBC done showing anemia (no MCV given) markedly raised WBC >200 Neutrophilia 141.7, eosinophilia 5.3, basophilia 6.4, Monocyte 3.2, Lymphocyte 10.7(all white cell differentials raised) Blast (3.2), promyelocyte (single digit), myelocyte (30 sth), metamyelocytes (double digit)
1. Most likely dx
2. 3 essential further Ix to confirm dx
3. Name 1 drug for tx
4. Doctor also prescribed febuxostat. What is the mechanism of action of febuxostat and how is it useful in treating the patient?

Answer 1

1. CML
2. Bone marrow aspirate and trephine biopsy
   Immunophenotyping (CD antigens)
   Molecular genetics
   Serum karyotyping for philadelphia chromosome translocation (t922)
   FISH for BCR ABL1
3. Imatinib (TKI)
4. Xanthine oxidase inhibitor (inhibits conversion of xanthine and hypoxanthine to uric acid) used to prevent tumor lysis syndrome, formation of uric acid stones

Question 2

45/F with metastatic ca ovary. Abd shifting dullness. One lower limb more swollen than the other (43 cm and 38 cm) not painful. SOB and desaturation to 94% O2, tachycardia. CXR clear. 60 kg body weight. Renal function normal.
1. 2 diagnostic imaging Ix to confirm dx of her chest condition
2. 2 common ECG findings
3. Immediate Mx. Prescribe the drug in clinical practise
4. If patient also complains of fever and abd pain. What Ix?
5. What genetic test. Specify the specimen

Answer 2

1. CTPA (VQ scan only done when renal function does not permit contrast), d-dimer (used to rule out PE), doppler USG lower limb, ECG
2. Sinus tachycardia. R heart strain, R axis deviation (P pulmonale), RBBB, S1Q3T3
3. Enoxaparin 1mg/kg SC BD 10 days
4. CBC for WBC, CRP, blood for culture and sensitivity testing, diagnostic paracentesis (D/C, culture and S/T)
5. BRCA1/2, tumor tissue

Question 3

Answer 3

a)
Sequestration
Destruction: sepsis/DIC
Production issue:
Marrow failure: aplastic aemia, meds, infection
Marrow infiltration: malignant (leukemia, multiple myeloma, MDS, metastasis), non malignant (fibrosis, infection)

ddx: aplastic anemia caused by chemotherapy

Question 4

Question 5

Question 6

A 26/F presents with a mediastinal mass on CXR. Biopsy shows Hodgkin lymphoma. She also has a palpable rubbery (?)axillary lymph node. She plans to start a family.
a. List 2 investigations for staging
b. Pathological hallmark of hodgkin lymphoma
c. List 2 social issues to discuss with her before treatment
d. She is being planned to receive ABVD. List 3 specific investigations to be done prior to giving adriamycin and bleomycin
She receives the treatment but later presents with a fever of 39 degrees and shortness of breath. CBC shows marked neutropenia and she is diagnosed with neutropenic fever.
e. List 2 management approach for neutropenic fever

Answer 6

a. PET scan, CT thorax, bilateral bone marrow aspirate and trephine biopsy
b. Reed sternberg cell (classical in nodular sclerosis type of HL)
c. Fertility (drugs affecting fertility), contraception (teratogenic chemotherapy drugs), finances if not HA sponsored drugs, ability for self care
d.
adriamycin (daunorubicin): ECG and echocardiogram
bleomycin: lung function test (causes interstitial lung disease), renal function test (before each cycle)
e. Empirical broad spectrum antibiotics: tazocin (piperacillin-tazobactam), meropenem, cefipime
G-CSF. IV fluid resuscitation.