Haemat SAQ Flashcards
42/F splenomegaly 5 cm below costal margin. Preliminary blood tests showed WBC 450, Hb 10.4, plt 500. Completely asymptomatic. No lymphadenopathy.
- Diagnosis (3)
- Ix
- Tx
6 months (years?) later, pancytopenia despite good drug compliance, and not taking other drugs. - What could be the cause of the presentation? (2)
- CML
- CBC with DC, peripheral blood smear, bone marrow aspirate and trephine biopsy, (morphology, cytochemistry (MPO/SBB), immunophenotyping (flow cytometry), cytogenetics (karyotyping, FISH for chromosomal abnormalities), molecular genetics (PCR for genetic mutations))
- TKI
- TKI myelosuppression
A 85-year-old woman with history of “abdominal surgery” 30 years ago.
Perioral vitiligo.
She was diagnosed to have vitamin B deficiency.
a) 2 causes of Vitamin B12 deficiency (2 marks)
b) Pathophysiology of how Vitamin B12 deficiency caused anaemia (2 marks)
c) 2 Cx of Vitamin B12 deficiency anemia
d) Two blood test to find the cause.
e) The patient was started on Vitamin B12 1000mcg for 3 days daily. Name some side effects of vitamin B12 replacement. (2)
a) gastrectomy, metformin, pernicious anemia, malabsorption due to short bowel, distal ileum surgical reseectiion
b) B12 is a cofactor in methylation of homocysteine into methionine which is important for methylation reactions includnig those that regulate DNA synthesis. Inadequate B12, bone marrow produces macrocytes due to improper cell division –> reduced RBC span contributing to anemia
c) subacute combined degeneration of spinal cord, peripheral neuropathy. Dementia, cerebellar degeneration, pancytopenia.
d) anti parietal cell and intrinsic factor antibodies.
e) injection site reaction, nausea, vomiting, headache
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?
- CML
- Bone marrow aspirate and trephine biopsy
Immunophenotyping (CD antigens)
Molecular genetics
Serum karyotyping for philadelphia chromosome translocation (t922)
FISH for BCR ABL1 - Imatinib (TKI)
- Xanthine oxidase inhibitor (inhibits conversion of xanthine and hypoxanthine to uric acid) used to prevent tumor lysis syndrome, formation of uric acid stones
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
- CTPA (VQ scan only done when renal function does not permit contrast), d-dimer (used to rule out PE), doppler USG lower limb, ECG
- Sinus tachycardia. R heart strain, R axis deviation (P pulmonale), RBBB, S1Q3T3
- Enoxaparin 1mg/kg SC BD 10 days
- CBC for WBC, CRP, blood for culture and sensitivity testing, diagnostic paracentesis (D/C, culture and S/T)
- BRCA1/2, tumor tissue
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
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
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.
A 32-year-old lady had prolonged bleeding after a dental extraction. Further investigations showed a normal platelet count, a normal prothrombin time but a prolonged activated partial thromboplastin time. Discuss the differential diagnosis.
ddx of prolonged bleeding and isolated increase in aPTT
* Heparin (heparin contamination/patient on heparin medication)
* von Willebrands disease (women: autosomal dominant)
* Factor inhibitor: F8 inhibitor (occasionally in patients with auoimmune idsease such as SLE (but bleeding does not occur unless there is coexistent severe ITP)
* Lupus anticoagulant: less likely as not associated with bleeding but thrombosis (do not inhibit coagulation factor)
An 80-year-old man presented with symptoms of anaemia, his blood counts showed Hb 6.5 g/dl, WCC 2.3 × 109/L and platelet count 82 × 109/L. Discuss the differential diagnoses and the important features to be notes on physical examination.
ddx of pancytopenia
* Bone marrow malignancy (leukemia, MDS)
* Bone marrow infiltration (secondary metastasis) such as CA lung or CA prostate
* Bone marrow failure (primary and secondary aplastic anemia, chemoirradiation)
* Hypersplenism
* Megaloblastic anemia (large platelets, hypersegmented neutrophil)
* Autoimmune: SLE
* Paroxysmal nocturnal haemoglobinuria
PE
* Pallor (anemia)
* jaundice (hemolysis as cause of anemia e.g. SLE)
* Petechiae, bruiss, conjunctival and retinal hemorrhage (features of thrombocytopenia)
* LN, tonsil, spleen and liver
* Dipstix: Hb
A 23-year-old young lady presented with sudden onset of painful left leg swelling. Ultrasonography confirmed deep vein thrombosis. Discuss the risk factors and the important features to be noted in the history.
- Prologed immobilization
- Drug induced: estrogens
- Pregnancy: increased coagulation factors
- Acquired conditions: malignancies, lupus anticoagulant, antiphospholipid syndrome
- Inherited conditions (AD): anti thrombin 2 deficiency, protein C deficiency, protein S deficiency
A 52-year-old woman with mitral valvular replacement was admitted with melaena. She also gave a history of joint pain. Her admission INR was 3.8. Discuss the causes of increased INR in her case.
Patient with MVC put on long term warfarin. INR used to monitor the warfarin. Increase in INR can be several reasons
* Taking a higher dosage than normal
* May have peptic ulcer and put on cimetidine (H2 blocker) which inhibits warfarin metabolism
* May also be HP+ve peptic ulcer and put on triple therapy which includes omeprazole and clarithromycin and metronidazole
* She may have gout and put on allopurinol. It can increase INR.
A 40-year-old lady presented with generalized bruising and easy gum bleeding for 1 week. Her platelet count was 15 × 109/L. A 3 cm splenomegaly was found on physical examination. Discuss the differen- tial diagnoses.
Decreased production of platelets
* Aplastic anemia
* Secondary bone marrow failure due to drugs/cytotoxic drugs
* MDS
* Myelofibrosis (fibrotic phase)
* Bone marrow infiltration by lymphoma, leukemia
Increased destruction of platelets: autoimmune disease e.g. SLE
Sequestration: portal hypertension leading to hypersplenism
A 50-year-old man was a heavy alcohol drinker. He was found to have a haemoglobin level of 9.2 gm/ dL. Discuss the likely causes of his anaemia.
Microcytic: iron deficiency anemia due to GIB (chronic: portal hypertensive gastropathy due to alcoholic cirrhosis, gastric erosion), (acute: variceal bleeding, mallory weiss tear due to severe vomiting after massive alcohol intake), thal trait
Macrocytic: B12 and folatel deficiency, alcohol, cirrhosis
Normocytic: pancytopenia (hypersplenism due to cirrhosis and portal hypertension), acute hemolysis in Zieve syndrome (transient jaundice, haemolytic anemia and hyperlipidemia associated with acute alcoholism in patients with cirrhosis or a fatty liver)
Primary cause of thrombocytosis: MPN diseases (ET, masked PV, myelofibrosis and CML)
Secondary/reactive cause of thrombocytosis: bleeding, chronic iron deficiency (GI bleeding), malignancy (CA lung), chronic infections e.g. TB. connective tissue diseases e.g. RA. Post splenectomy
Ix
* CBC (look at MCV for iron deficiency anemia as a cause of thrombocytosis, haematocrit may be raised in MPN, persistent increase in platelet count of >6000x10^9/L is a central diagnostic feature of essential thrombocythemia)
* Serum ferritin (exclude iron deficiency anemia) to distinguish from PV (high Hb, haematocrit, normal hb with reactive thrombocytosis –> a masked PV)
* Peripheral smear (exclude small fragmented RBCs misinterpreted by automated haematology counters as platelets and falsely elevated count
Test to exclude reactie thrombocytosis
* PPD test for possible TB
* CXR to exclude TB, CA lung
* CT scan for suspected intraabd malignancy
If reactive thrombocytosis ruled out, bone marrow biopsy may be necessary to look for a primary cause
(a) pernicious anemia, clinical signs (pallor, glossitis, vitiligo), blood picture (macrocytosis (high MCV) with anemia, low white cell and platelet)
(b) reticulocyte count, vit B12 and folate assay: low vitamin B12 in pernicious anemia due to lack of intrinsic factor for absorption. Serum methylmalonic acid and homocysteine levels both elevated in vit B12 deficiency. PBS: hypersegmented neutrophils.
Bone marrow examination: exclude MDS. Pernicious anemaei: OGD for atrophic gastritis, autoantibodies against parietal cels
(c) IM B12 with regular FU for Hb and reticulocyte count monitoring
(a) macrocytic anemia
(b) high LDH, high total bilirubin
(c) haemolytic anemia
(d) spherocytosis, polychromasia
(e) direct coombs test
(f) small lymphoatic lymphoma (most common association)
(a) pancytopenia, prolonged aPTT, abnormal promyelocyte
(b) life threatening bleeding from DIC
(c) immediate ATRA and ATO + dexa prophylaxis, platelet and FFP
(d) APML
(e) Bone marrow aspirate and trephine biopsy. Karyotyping for t15;17, FISH for PML/RARa
(f) Induction by ATRA/ATO. ATRA/chemo (high risk). post remission therapy by ATRA/ATO or ATRA/anthracycline
(a) thal intermedia
(b) hemoglobin studies (supravital stain for HbH, Hb profile)
(c) hemolysis due to splenomegaly and presence of insoluble tetramers
(d) high serum Fe and transferrin saturation
(e) serum Fe is an acute phase reactant. Chronic ineffective erythropoiesis can lead to increased iron absorption and iron overload this may be present even in those who have not recieved any transfusion before. Especially can be adult onset iron overload in thal intermedia
