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HIS > HIS24 Myelodysplatic Syndrome (MDS) And Myeloproliferative Neoplasm (MPN): Principles Of Diagnosis And Management > Flashcards

HIS24 Myelodysplatic Syndrome (MDS) And Myeloproliferative Neoplasm (MPN): Principles Of Diagnosis And Management Flashcards

1
Q

Classification of Myeloid neoplasms and Acute leukaemia

A
  1. ***MPN
  2. ***MDS
  3. ***MDS/MPN
  4. ***AML and related precursor neoplasms
  5. Mastocytosis
  6. Myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, FGFR1, PCM1-JAK2
  7. Myeloid neoplasm with germline predisposition
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2
Q

Myelodysplastic syndrome (MDS)

A

Clonal (neoplastic, from 1 cell) haematological disorder associated with ineffective haematopoiesis, cytopenia (NOT proliferative), propensity of clonal progression to AML

Ineffective haematopoiesis: 記住: ***Hypercellular BM but Cytopenia

Key features:

  1. Cytopenia >= 1 lineage (Erythroid, Myeloid, Megakaryocytes)
  2. Hypercellular BM —> but abnormal/dysplastic cells undergo premature apoptosis —> Cytopenia
  3. Morphologic dysplasia >= 1 lineage
  4. +/- Increase in blasts of myeloid lineage (<20%) —> immature cells / blasts may increase —> ***progress to AML

Male preponderance (2:1) with incidence increasing after 5th decade of life (median age at presentation ~75 yo)

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3
Q

MDS typical presentation

A
  • Incidental finding of cytopenia >=1 lineage
  • Symptoms / Complications in relation to cytopenia >=1 lineage
  • In symptomatic patients, ~90% have significant Macrocytosis (>100 fl) at presentation
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4
Q

Classification of MDS

A

Low-grade MDS (Dysplasia without excess blasts)

  • ***blast <5% (BM, PB)
  • ***no high risk karyotype / cytogenetics
  • indolent in presentation
  • main problem is cytopenia
  • lower risk of progression to AML (usually <10%)
  • survival variable depending on subtype

High-grade MDS (Dysplasia with excess blasts and/or high risk cytogenetics)

  • ***blast 5-19% (BM, PB)
  • ***high risk karyotype / cytogenetics
  • more symptomatic (more severe cytopenia)
  • high risk progression to AML within 2 years (>50%) —> Pre-leukaemia
  • poor long term survival
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5
Q

Aims of investigations for MDS

A
  1. Exclude alternative and reversible causes (of cytopenia)
  2. Confirm diagnosis - demonstrate ineffective haematopoiesis + dysplasia +/- increase in blasts + ***confirm clonality (genetics / molecular)
  3. Prognosis
  4. Fitness to treatment, esp. for high risk patients
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6
Q

***Investigations for suspected MDS

A
  1. CBC with blood film review + D/C
    - look for dysplasia, cytopenia
  2. Reticulocyte count
    - often low
  3. Serum active vit B12, folate (serum + red cell)
    - excluded B12, folate deficiency
  4. LFT, RFT, LDH
  5. Other investigations: depending on clinical context guided by history, physical examinations depending on preliminary CBC and other findings
    - e.g. exclude end-stage liver disease
Diagnostic investigations (after excluding obvious alternative causes):
1. BM aspiration, Trephine biopsy
  1. BM aspiration for cytogenetic, molecular studies —> to prove clonality
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7
Q

Prognostic factors for MDS

A
  1. ***Cytopenia severity
  2. ***BM blast %
  3. ***Cytogenetics (Karyotype + Gene mutations)
  4. Performance status
  5. Timely treatment and good supportive care
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8
Q

***Principles of treatment of MDS

A

Low-grade MDS / R-IPSS very low to intermediate risk MDS
1. Supportive (e.g. transfusion)
2. Erythropoietin-stimulating factors (ESA)
3. G-CSF (for Granulopoiesis)
—> main aim to stimulate erythropoiesis

(- Lenalidomide for 5q deletion syndrome (rare)
- TGFβ targeting (Luspatercept))

High-grade MDS / R-IPSS intermediate to very high risk MDS
1. **Hypomethylating agents (Azacitidine, Decitabine)
—> only prolong survival and delay progression to AML
2. **Allogeneic HSCT in young and transplant-eligible patients
—> only modality proven to have long term cure

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9
Q

Hypomethylating / Demethylating agents MOA

A

Pathogenesis:
Abnormal DNA methylation
—> Inactivation of tumour suppressor genes / genes responsible for transcription
—> Leukaemogenic / Stopped transcription

MOA:
Decitabine/Azacitidine
Incorporate into DNA/RNA respectively
1. ***Inhibit DNA methyltransferase
—> Induce DNA ***hypomethylation
—> ***Reactivation of silenced genes (esp. tumour suppressor genes)
  1. Block protein, DNA synthesis
    —> ***Cytotoxicity
  2. Block DNA synthesis
    —> ***Inhibit cellular proliferation
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10
Q

Case 1:

  • 80 yo
  • retired teacher, non-smoker, non-drinker
  • hypertension, hyperlipidaemia well controlled
  • fresh per-rectal bleeding, anaemic symptoms for 4 weeks
  • Hb: 5.1
  • WBC: 1.8
  • Platelet count: 56
  • MCV: 115
  • last blood test in 2016 showed normal blood counts
  • pallor
  • LN, liver, spleen not palpable
  • external haemorrhoids on per-rectal exam
A
  • Hb: 5.1 —> Low
  • WBC: 1.8 —> Low
  • Platelet count: 56 —> Low
  • MCV: 115 —> High (Macrocytosis)
  • 2016 normal —> Acquired condition
  • Over a month —> Insidious
  • LN, liver, spleen not palpable —> ***Consistent with MDS

Differential diagnosis of Pancytopenia with significant Macrocytosis (MCV>110):

  1. ***MDS
  2. ***Pernicious anaemia (a type of Megaloblastic anaemia, organ-specific autoimmune disease —> Ab against intrinsic factor for B12 absorption in terminal ileum —> B12 deficiency)
  3. Acute leukaemia (occasionally) (esp. elderly AML / AML with MDS-related changes)

Investigations:
1. PB blood film
- RBC:
—> Macrocytosis
—> Absent Polychromasia (i.e. Recticulocytopenia —> production problem)
- WBC:
—> Leukopenia
—> Hypolobated neutrophils, No hypersegmented neutrophils (***Hypersegmented —> B12 folate deficiency)
—> Hypogranular neutrophils
—> Pseudo Pelger-Huet anomaly
—> Abnormally lobulated neutrophil
—> Circulating blasts (2%) with no Auer rod
- Platelets:
—> Thrombocytopenia with no platelet clumps
- Conclusion: Pancytopenia + Dysplastic features + Occasional circulating blasts
3. LRFT, LDH —> Normal
4. Clotting profile —> Normal
5. Serum active B12, Serum and Red cell folate —> Normal

Diagnostic investigation:
1. BM aspirate
- Markedly hypercellular BM
- Erythroid:
—> Erythropoiesis increased
—> Marked N:C asynchrony (usually synchronised development)
—> Ring sideroblasts on Fe stain (abnormal incorporation of Fe / abnormal Fe complex)
—> Bilobed Erythroid cells (usually 1 single nuclei)
- Myeloid:
—> Hypolobated / Abnormally lobated myeloid series (4%)
- Megakaryocytes:
—> Micromegakaryocytes
—> Separate nuclei + Hypolobated (usually hyperlobated)
- Conclusion: Hypercellular BM, Trilineage dysplasia, Leukaemic blasts

Diagnosis:
- MDS (∵ Cytopenia + Hypercellular BM) with multilineage dysplasia and ring sideroblasts

Further investigation:
- Cytogenetics —> Complex karyotype with del(5)(q13q33), del(9)(q22q32), -7, -13, -14
—> very poor risk by R-IPSS

Treatment:
- Started on Azacitidine in view of good performance status

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11
Q

Differential diagnosis of Pancytopenia with significant Macrocytosis (MCV>110)

A
  1. ***MDS
  2. ***Pernicious anaemia (a type of megaloblastic anaemia, organ-specific autoimmune disease —> Ab against intrinsic factor for B12 absorption in terminal ileum —> B12 deficiency)
  3. Acute leukaemia (occasionally) (esp. elderly AML / AML with MDS-related changes)
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12
Q

Myeloproliferative neoplasm (MPN)

A

Clonal HSC disorder characterised by **proliferation of >=1 haematopoietic lineages in BM/PB
—> **NO premature apoptosis (vs MDS)
—> ***NO cytopenia

Key features:

  1. Increased circulating cells
  2. Hypercellular BM
  3. Absence of myelodysplastic features

Cause:
- Driver mutation / Gene fusion activate ***Tyrosine kinases (e.g. JAK kinase) + Cytokines signalling

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13
Q

Classification of MPN

A

Philadelphia chromosome +ve:
**1. Chronic myeloid leukaemia
- defined by **BCR-ABL1-positive (t(9;22)(q34.1;q11.2): ***Philadelphia chromosome +ve) (BCR-ABL-1-negative does not exist!!!)
—> translocation of gene from chromosome 22 to 9

(100% of CML with BCR-ABL1-positive)

Philadelphia chromosome -ve MPNs:

  • **2. Polycythaemia Vera (JAK2 V617F)
  • **3. Primary myelofibrosis (JAK2, CALR, MPL)
  • **4. Essential thromobocythaemia (JAK2, CALR, MPL)
    5. Chronic neutrophilic leukaemia
    6. Chronic eosinophilia leukaemia, NOS
    7. MPN, unclassifiable

(***ALL can progress to AML!!!)

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14
Q

CML, BCR-ABL1+

A

***Triphasic disease (defined by blast % in BM/PB):
1. Chronic phase (CP) (<10%)
2. Accelerated phase (AP) (10-19%)
3. Blast phase (BP) (>20%)
—> AP, BP rarely seen in modern era due to Tyrosine kinase inhibitors

CP:

  • 50% asymptomatic at presentation
  • Fatigue, weight loss, fever
  • ***Splenomegaly
  • CBC findings:
    —> Leukocytosis (very high)
    —> Left shift with entire myeloid series on PB + **Bimodal prominence of **Neutrophils and **Myelocytes
    —> **    Absolute Basophilia
    —> **Anaemia (intense proliferation of myeloid / megakaryocytes lineage —> suppressed erythropoiesis)
    —> **    Thrombocytosis
    —> Blasts not increased (<2%)
  • BM findings:
    —> Hypercellular with increased Myeloid:Erythroid ratio
    —> Blasts <10%
    —> Entire myeloid series with ***Bimodal prominence
    —> Increased small + hypolobated megakaryocytes
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15
Q

Diagnostic investigations for CML

A
  1. BM, PB morphology
  2. Cytogenetic, FISH (for t(9;22): ABL: chromosome 9, BCR: chromosome 22)
  3. Quantitative real-time PCR (to detect BCR-ABL1 for diagnosis and disease monitoring)
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16
Q

***Treatment of CML

A
  1. Tyrosine kinase inhibitors (x rmb):
    - 1st generation: ***Imatinib
    - 2nd generation: Nilotinib, Dasatinib
    - 3rd generation: Ponatinib (only for T315l kinase domain mutation)

MOA:
Inhibitor of BCR-ABL1
—> binds to kinase domain (ATP binding domain) of ABL1
—> ATP cannot bind
—> cannot donate phosphate group for subsequent effector function, proliferation

  1. Allo-HSCT
    - only indicated in AP, BP of CML
17
Q

Polycythaemia Vera

A
  • Slight male preponderance
  • Median age 55-60

***記: Leukocytosis + Polycythaemia + Thrombocytosis

CBC (***Panmyelosis: ↑ ALL 3 lineages):

  • ↑ Hb, Haematocrit, RBC
  • ↑ WBC
  • ↑ Platelet

Cytogenetics: JAK2 V617F

Presentations:
1. Fatigue, Aquagenic pruritis (∵ cytokines ↑)

  1. Mild - moderate splenomegaly (70%) + hepatomegaly (40-50%)
  2. Secondary to vascular disturbances and ↑ red cell mass (e.g. numbness of hands, feet due to microvascular obstruction, arterial thrombosis, stroke, AMI, VTE, embolisms)
  3. Gout (high uricaemia due to high cell turnover, uric acid release from cells)

—> May progress into secondary Myelofibrosis (Post-PV MF)

18
Q

Essential thrombocythaemia

A
  • Another peak in women at 30
  • Median age 50-60
  • 30-50% asymptomatic, present with incidental finding of abnormal blood counts

***記: 齋Thrombocytosis

CBC:

  • Marked isolated ***Thrombocytosis + Platelet anisocytosis
  • Hb normal
  • WBC usually normal / mildly ↑

Cytogenetics: JAK2, CALR, MPL

  • Symptoms:
    1. Vascular disturbances
  1. **Thrombotic / haemorrhagic episodes (↑ platelet dysfunction, abnormal platelet adhesion, **consumption of von Willebrand factor —> ***aVWD)
  2. Mild splenomegaly occur in 15-20%

—> May progress into secondary Myelofibrosis (Post-ET MF)

19
Q

Primary myelofibrosis

A
  • Both genders affected
  • Median age: 65-70
  • Insidious onset
  • Symptoms depend on phase of disease

***記: Leukocytosis + Anaemia + Thrombocytosis

CBC:
- Leukocytosis (**Myeloid)
- **Anaemia
- Thrombocytosis (Megakaryocytes) (late stage MF: Thrombocytopenia)
- **Leukoerythroblastic blood picture (∵ Fibrotic BM —> Proliferation of myeloid cells —> push immature WBC + RBC into PB)
—> **Tear-drop poikilocytes (RBC pass through abnormal BM sinusoids / splenic sinusoids —> squeeze out)
- Late stage: Pancytopenia

Cytogenetics: JAK2, CALR, MPL

Features:

  1. Clonal proliferation —> Splenomegaly, Anaemia
  2. ***Bone marrow stromal changes (∵ stimulation of marrow fibrosis) —> Coarsening of reticulin fibres + Collagen fibrosis + Thickened trabecular bone
  3. Secondary inflammatory state (∵ ***Hypercytokinemia) —> Constitutional symptoms, cachexia

Presentation:

  1. ***↑ BM fibrosis —> suppress erythropoiesis
  2. ***Constitutional symptoms, Anaemic symptoms, Massive splenomegaly (∵ Hypercytokinemia)
  3. ***Hypercytokinemia —> suppress erythropoiesis
  4. ***Markedly ↑ LDH (∵ cellular proliferation)
20
Q

Aims of investigations for PV, ET, MF

A
  1. Morphologic diagnosis by PB and BM
  2. Proof of clonality (by cytogenetic, molecular studies)
  3. Exclusion of CML BCR-ABL1+ (some CML have isolated thrombocytosis)
  4. Exclusion of reactive causes (esp. for ET/MF without somatic mutations)
  5. Prognostic assessment (***risk of thrombosis in PV, ET, survivals for MF)
21
Q

***Investigations for MPN

A

General investigations

  1. History, P/E (spleen, liver size, assessment of thrombotic/haemorrhagic events, CV risk factors, medical history)
  2. CBC with D/C + Blood film review
  3. LRFT, LDH, urate
  4. EPO level (suppressed in PV)
  5. Clotting profile + Exclusion of aVWD (esp. extreme thrombocytosis with bleeding tendency)
  6. Hep B, C serology (for treatment)
  7. CXR
  8. Investigations to exclude reactive causes

Diagnostic investigations

  1. BM exam, Trephine biopsy
  2. Cytogenetic analysis
  3. Molecular studies (JAK2, CALR, MPL, BCR-ABL1 to exclude CML)
22
Q

Risk factors for thrombosis in PV and ET

A

High risk of thrombosis in PV:

  • Age >60
  • History of thrombosis
  • CVS risk factors

High risk of thrombosis in ET:

  • Age >60
  • History of thrombosis
  • CVS risk factors
  • JAK2V617F
23
Q

PMF risk assessment

A

Genetically-inspired IPSS (GIPSS)

Other systems:
MIPSS70

24
Q

MPN disease burden and treatment

A
  1. Vascular complications (i.e. risk of thromboembolism)
    - Antiplatelet for managing risk
    - Cytoreduction for maintaining normal Hct, platelet etc.
  2. Organomegaly + Symptoms
    - JAK/STAT inhibition (Ruxolitinib) (esp. PMF ∵ most symptomatic)
    - Cytoreduction (Hydroxyurea and IFN)
  3. Disease progression and survival
    - JAK/STAT inhibition (Ruxolitinib)
    - Cytoreduction (Hydroxyurea and IFN)
    - Allo-HSCT (***PV, ET does NOT require HSCT unless progress to MF)
25
Q

MOA of Ruxolitinib in MF

A 
JAK2 inhibitor
—> inhibit Clonal proliferation + Cytokine proliferation
1. Control constitutional symptoms
2. Control organomegaly
3. Stabilising marrow fibrosis
26
Q

Treatment of Low-risk and High-risk PV

A

Low-risk PV:

  1. Low-dose aspirin
  2. Phlebotomy (Venesection) to maintain Hct <45%
  3. Manage CVS risk factors
  4. Monitor for new thrombosis / bleeding
  5. Evaluate indications for cytoreductive therapy
  6. Monitor signs/symptoms of disease progression

High risk PV:

  1. Low-dose aspirin
  2. Phlebotomy to maintain Hct <45%
  3. Manage CVS risk factors
  4. Monitor for new thrombosis / bleeding
  5. Hydroxyurea / Peg-IFNα 2A (suppress proliferation, immunomodulation, promote apoptosis) (based on age and patient-specific variables)
  6. Monitor for response and signs/symptoms of disease progression
  7. Adequate response —> continue treatment
  8. Inadequate / loss of response —> change of cytoreductive therapy
27
Q

Treatment of very low, low, intermediate, high-risk ET

A

Very low, Low, Intermediate risk ET:

  1. Aspirin
  2. Manage CVS risk factors
  3. Monitor for new thrombosis, disease-related major bleeding
  4. Monitor for acquired vWD
  5. Monitor signs/symptoms of progression
  6. Evaluate indications of cytoreductive therapy
  7. Asymptomatic —> continue Aspirin
  8. Symptomatic with indications of cytoreduction —> start cytoreductive agents

High-risk ET:

  1. Aspirin
  2. Manage CVS risk factors
  3. Monitor for new thrombosis, disease-related major bleeding
  4. Monitor for acquired vWD
  5. Monitor signs/symptoms of progression
  6. Hydroxyurea / Peg-IFNα 2A +/- Anagrelide (inhibit megakaryocytes maturation) (based on age and patient-specific variables, disease factors)
  7. Adequate response —> continue treatment
  8. Inadequate / loss of response —> change of cytoreductive therapy
28
Q

Proposed algorithm for PMF treatment

A

MF with prognosis assessed (by clinical, molecular studies)

  1. Predicted long term survival, Low risk AML
    - Ruxolitinib (based on symptoms, organomegaly)
    - Cytoreduction
    - Anaemia, Cytopenia management
  2. Predicted short median survival, High risk AML
    - Allo-HSCT if transplant eligible
    - Ruxolitinib (based on symptoms, organomegaly)
    - Cytoreduction
    - Anaemia, Cytopenia management
29
Q

Case 2:

  • 80 yo man
  • ex-smoker, 40 pack-years, non-drinker
  • hypertension well controlled with 1 antihypertensive
  • dizziness and headache for 2 weeks
  • generalised pruritis, worse after taking bath (Aquagenic pruritis)
  • Hb 19.2
  • MCV 80
  • WBC 19
  • platelet 725
A
  1. CBC + D/C + PB film
    —> packed
    —> Leukocytosis with left-shift, occasional myelocytes
    —> Thrombocytosis with platelet anisocytosis
  2. Biochemistry
    - LRFT normal
    - LDH ↑ at 800
    - Urate ↑
    - EPO markedly suppressed
  3. BM aspirate, Trephine biopsy
    - Marked trilineage hyperplasia (Panmyelosis)
    - Pleomorphic megakaryocytes
    - Absent marrow fibrosis
  4. Suggestion: PV
  5. Molecular study, Cytogenetics
    - JAK V617F mutation
    - Cytogenetics: Normal
  6. Treatment
    - Aspirin
    - Venesection (放血)
    - Hydroxyurea
30
Q

Differential diagnoses of genuine erythrocytosis

A

Primary (endogenous EPO level suppressed by -ve feedback):
- PV (MPN)

Secondary (EPO level ↑ / NOT suppressed):

  1. Chronic hypoxaemic states (e.g. chronic lung diseases, OSA, cyanotic congenital heart disease)
  2. Renal conditions with increased EPO production (e.g. polycystic kidney disease, renal cell carcinoma)
  3. Rare paraneoplastic states (e.g. RCC, HCC)
  4. Others (e.g. smoking)
31
Q

Case 3:

  • 28 yo female
  • good past health
  • heavy menses
  • pain and numbless over hands
  • Hb 12.9
  • MCV 90
  • WBC 9.12
  • platelet 1126
  • Afebrile
  • No palpable LN
  • liver, spleen not palpable
  • other systems unremarkable
A
  1. CBC + D/C + PB film
    - Marked isolated thrombocytosis
    - Platelet anisocytosis (many giant platelets)
    - No Leukocytosis
    - No Basophilia
    - No blasts in circulation
    - No tear-drop poikilocytes
    - No hypochromic, microcytic RBC —> NOT Fe deficiency
  2. Biochemistry
    - LRFT, LDH, urate normal
    - Fe profile —> normal —> NOT Fe deficiency
    - PT 12.4s (10.9-13.6), APTT 36s (25.1-33.9)
  3. BM aspirate, Trephine biopsy
    - Marked megakaryocytic hyperplasia with large and hyperlobated megakaryocytes
    - No evidence of marrow fibrosis
    - Erythroid, Myeloid series unremarkable
  4. Suggestions: ET
  5. Molecular studies, Cytogenetics
    - CALR exon 9 mutation —> proving clonality
    - Cytogenetics —> normal
    - RT-PCR for BCR-ABL1 —> negative
  6. Treatment
    - Peg-IFNα 2A for cytoreduction
    - Aspirin for prevention of thromboembolism
    - not consider hydroxyurea because teratogenic and young age, possibility of secondary malignancy
32
Q

Differential diagnoses of isolated thrombocytosis

A

Primary

  • ET (MPN)
  • CML BCR-ABL1+ (rare) (MPN)

Secondary (reactive) (Thrombocytosis rarely extreme)

  • Bleeding / Fe deficiency (most common)
  • Paraneoplastic phenomena
  • Inflammation (sub-acute/chronic infections, autoimmune diseases) (rare)
33
Q

Case 4:

  • 58 yo female
  • reduced exercise tolerance, weight loss, loss of appetite, abdominal discomfort for 6 months
  • pallor
  • palpable liver 5cm below right costal margin
  • enlarged spleen to umbilicus
  • Hb 7.6
  • WBC 43.37
  • Platelet 550
A
  1. CBC + D/C + PB film
    - marked RBC anisopoikilocytosis
    - prominent tear-drop poikilocytes
    - Leukocytosis with left-shift
    - thrombocytosis with many giant platelets
    - occasional nucleated RBC, myelocytes, blasts (all immature cells) —> Leuko-erythroblastic blood picture
  2. Biochemistry
    - LRFT normal
    - LDH ↑ at 1100
    - urate ↑
  3. BM aspirate, Trephine biopsy
    - BM aspirate —> dry tap
    - Trephine biopsy
    —> streaming artefacts
    —> ↓ erythropoiesis
    —> ↑ granulopoiesis
    —> megakaryopoiesis
    —> megakaryocytes showed nuclear atypia, clustering, abnormal topographic distributed
    —> Reticulin staining showed marked coarsening of reticulin fibres with many intersections and focal collagen fibrosis (i.e. severe BM fibrosis)
    —> thickened trabecular bone
  4. Suggestion: PMF
  5. Cytogenetics, molecular studies
    - Cytogenetics: Normal
    - Molecular studies: JAK2 V617F
    - Next-generation sequencing showed high risk mutations (ASXL1, EZH2, SRSF2 mutations)
  6. Treatment
    - Ruxolitinib for symptom and splenomegaly control
    - Allogeneic HSCT from sibling donor in view of very high risk disease
34
Q

MDS/MPN

A

Clonal haematological disorders characterised by

  • proliferation in 1 lineage (typically leukocytosis) —> ↑ Circulating cell + Hypercellular marrow
  • cytopenia in >=1 other lineage(s) (typically anemia / thrombocytopenia)
  • dysplasia in >=1 lineage

Presentation very similar to MDS apart from proliferative element

Example: Chronic myelomonocytic leukaemia (CMML)

  • **CMML typically present in elderly with
  • Leukocytosis
  • Monocytosis
  • +/- Anaemia (Dysplastic change in Erythroid lineage)
  • +/- Thrombocytopenia (Dysplastic change in Megakaryocyte lineage)

Investigations, Therapy similar to MDS

35
Q

Case 5:

  • 79 yo man
  • anaemic symptoms
  • easy bruising
  • pallor
  • hepatosplenomegaly
  • Hb 6
  • WBC 35
  • platelet 45
A
  1. CBC + D/C + PB film
    - Monocytosis
    - Occasional promonocytes
    - Dysplastic neutrophils (hypogranular with abnormal lobulation)
  2. BM exam
    - Markedly ↑ Myeloid series, Monocytosis
    - Dysplastic Myeloid series + Megakaryocytes
  3. Diagnosis: CMML

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