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Y5 Haematology > Myelodysplastic Syndromes + Aplastic Anaemia > Flashcards

Myelodysplastic Syndromes + Aplastic Anaemia Flashcards

1
Q

Clinical Features of Myelodysplastic Syndromes

A

BM failure and Cytopenias: infection, bleeding, fatigue
Hypercellular BM
Defective cells (RBC, WBC, Platelets) - expanded in later Q

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2
Q

How are RBC’s defective in Myelodysplastic Syndromes?

A

There is dyserythropoiesis of RBC: may see RING SIDEROBLASTS (abnormally nucleated blast surrounded by iron granule ring)

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3
Q

How are WBC’s defective in Myelodysplastic Syndromes?

A

Myelokathexis of neutrophils and precursors: hypogranulation, Pseudo-Pelger-Huet anomaly (bilobed/hyposegmented neutrophils)

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4
Q

How are platelets defective in Myelodysplastic Syndromes?

A

Dysplastic megakaryocytes with hypolobated nuclei, micromegakaryocytes

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5
Q

What are Myelodysplastic Syndromes?

A

A heterogenous group of progressive disorders featuring ineffective proliferation and differentiation of abnormally maturing myeloid stem cells.
Characterized by: peripheral cytopenia, qualitative abnormalities of cell maturation; risk of AML transformation

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6
Q

What age group do Myelodysplastic Syndromes typically affect and how quickly do symptoms develop?

A

Elderly; symptoms usually develop over weeks/months

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7
Q

What 3 factors contribute to the prognosis of Myelodysplastic Syndromes?

A

BM blast % (lower is better), karyotype, degree of cytopenia

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8
Q

What is the Mortality rule of 1/3 for Myelodysplastic Syndromes?

A

1/3 die from infection, 1/3 die from bleeding, 1/3 die from Acute Leukaemia

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9
Q

What percentage of patients with Myelodysplastic Syndromes develop Acute Myeloid Leukemia?

A

50%

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10
Q

When does a Myelodysplastic Syndrome become AML? (in relation to blast %)

A

> 20% blasts = AML

under 5% = normal

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11
Q

What are the treatments for Myelodysplastic Syndromes?

A

Supportive: transfusion, SPO, G CSF, ABx
Biological modifiers: immunosuppressive drugs, Lenalidomide, Azacytidine (hypomethylating agent)
Chemotherapy: Hydroxyurea/Carbamide, Cytarabine
Allogenic SCT (Stem Cell Therapy)

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12
Q

What are the subtypes of Myelodysplastic Syndromes, in order of progression? (May just be easier to look at the table on pg22 for this bit!)

A

Refractory Anaemia, Refractory Anaemia with Ringed Sideroblasts (RA +RS), Refractory Cytopenia with multilineage dysplasia (RCMD), RCMD + RS, Refractory Anaemia with excess blasts (RAEB I), RAEB II, RAEB III, MDS with 5q deletion, MDS Unclassified.

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13
Q

Blood and Bone Marrow features for Refractory Anaemia (RA)

A

Blood: Anaemia, no blasts

Bone Marrow: Erythroid dysplasia with less than 5% blasts

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14
Q

Blood and Bone Marrow features for Refractory Anaemia + Ringed Sideroblasts (RA + RS)

A

Blood: Anaemia, no blasts

Bone Marrow: Erythroid dysplasia with >15% ringed sideroblasts

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15
Q

Blood and Bone Marrow features for Refractory Cytopenia with multilineage dysplasia (RCMD)

A

Blood: Cytopenia in >= 2 cell lines

Bone Marrow: Dysplasia in > 10% cells in >= 2 cell lines

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16
Q

Blood and Bone Marrow features for RCMD + RS

A

Blood: Cytopenia in >= 2 cell lines

Bone Marrow: Dysplasia in >10% cells in >= 2 cell lines + >15% ringed sideroblasts

17
Q

Blood and Bone Marrow features for RAEB I

A

Blood: Cytopenias, less than 5% blasts, no auer rods

Bone Marrow: Dysplasias, 5-9% blasts

18
Q

Blood and Bone Marrow features for RAEB II

A

Blood: Cytopenias or 5-9% blasts, or Auer rods

Bone Marrow: Dysplasias; 10-19% blasts or Auer rods

19
Q

Blood and Bone Marrow features for MDS with 5q deletion

A

Blood: Anaemia, normal or increased platelets

Bone Marrow: megakaryocytes with hypolobated nuclei and less than 5% blasts

20
Q

Blood and Bone Marrow features for MDS Unclassified

A

Blood: Complex - cytopenias, no blasts, no Auer Rods

Bone Marrow: Complex - myeloid or megakaryocytic dysplasia, less than 5% blasts

21
Q

What is Aplastic Anaemia?

A

The inability of BM to produce adequate blood cells.
Typically refers to deficient RBC’s only, but patients can have a pancytopenia as well.
Hypocellular BM: Haemaopoeitic stem cell numbers are reduced in BM trephines

22
Q

What are the symptoms of Aplastic Anaemia?

A

Anaemia: tiredness, palor, AF, SOB etc.
Leucopenia: increased infections
Thrombocytopenia: easy bruising + bleeding problems

23
Q

Aplastic Anaemia is Split into Primary and Secondary. What are the 2 subsections of primary, and which is more common? What percentage of AA is secondary?

A

Primary: Idiopathic (70%), Inherited (10%)
Secondary: 10 - 15%

24
Q

Causes of Secondary Aplastic Anaemia

A

Malignant infiltration, Radiation, AI (SLE), Drugs (Chemo), Viruses (Parovirus, Viral Hep.), Benzene chemicals

25
Q

4 main Inherited Aplastic Anaemias and method of inheritance. Which condition is the most common.

A

Fanconi Anaemia - Autosomal recessive
Dyskeratosis - X linked recessive (sometimes AR or AD)
Schwachman-Diamond Syndrome - Autosomal Recessive
Diamond Blackfan Syndrome - Autosomal Dominant with incomplete penetrance

Most common = Fanconi Anaemia

26
Q

Other than AA, what are the DDx of Pancytopenia with hypocellular marrow?

A

Hypoplastic MDS/AML; Hypocellular ALL; Hairy Cell Leukaemia; Atypical Mycobacterial Infection; Anorexia Nervosa/Starvation; Idiopathic Thrombocytopenic Purpura

27
Q

What are the Camitta Criteria for Severe Aplastic Anaemia?

A

Bone Marrow less than 20% cellularity + 2/3 peripheral blood features:
Reticulocytes under 1%
Neutrophils under 0.5%
Platelets under 20

28
Q

Clinical Triad of Dyskeratosis Congenita

A

Skin pigmentation, nail dystrophy, oral leukoplakia

90% will also have BM failure

29
Q

Somatic presentations of Fanconi Anaemia (found in 60-70% of cases)

A

Skeletal abnormalities (small thumbs), short stature, renal malformations, microopthalmia, skin pigmentation (café au lait spots, hypopigmented spots), microcephaly, hypogonadism, congenital heart defects

30
Q

What 2 conditions are patients with Fanconi Anaemia at high risk of getting, and what percentage do get the conditions?

A

Myelodysplastic Syndrome - 30%

AML - 10%

31
Q

When does Fanconi Anaemia usually present?

A

5-10 yrs

32
Q

What is the predominant chromosomal pathology in Dyskeratosis Congenita?

A

Chromosomal instability: telomere shortening

33
Q

What clinical symptoms may be present in Schmachman-Diamond Syndrome?

A

Neutrophilia - main presentation

Skeletal abnormalities, endocrine and pancreatic dysfunction, hepatic impairment, short stature

34
Q

What is Diamond-Blackfan Syndrome and when does it present?

A

Pure red cell aplasia: normal WCC and platelets

Presents at 1yr/neonatal

Y5 Haematology (10 decks)

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