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HEMATOLOGY > PANCYTOPENIA > Flashcards

PANCYTOPENIA Flashcards

1
Q

Define pancytopenia.

A

def. of BLOOD cells of ALL lineages

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2
Q

Is pancytopenia a dx?

Does it always mean bone marrow failure?

A

NO

-NO

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3
Q

Describe the maturation of a red cell.

A
  1. Pronormoblast
  2. Early normoblast
  3. Intermeidate normoblast
  4. Late normoblast
  5. Reticulocyte
  6. Mature Red cell
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4
Q

Describe the sequence of neutrophil maturation.

A 
Myeloblast
Promyelocyte
Myelocyte
Metamyelocyte
Band neutrophil
Segmented neutrophil
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5
Q

Describe the seq. of platelet maturation

A

Myeloid stem cell
Megakaryoblast
Megakaryocyte

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6
Q

Why may pancytopenia occur?

A
  1. INCREASED DESTRUCTION

2. REDUCED prodn

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7
Q

What causes reduced prodn?

A
  • bone marrow failure (INHERITED syndromes)

- acquired (Iary or IIary)

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8
Q

Why may bone marrow failure occur?

A
  1. cancer pre-disposition
  2. impaired haemopoiesis
  3. congenital anomalies

all d.t DEFECTS in DNA repair/ribosomes

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9
Q

How may inherited marrow failure present in an individual?

A

(rare)
- short
- skin problems
- radial ray abnormalities
- hypogenitilia
- endocrinopathies
- GI defects
- renal and hemato issues

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10
Q

Name an inherited marrow failure syndrome.

A

Fanconi’s Anaemia

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11
Q

What are radial ray abnormalities?

A
  • range of upper limb anomalies

- –radial HYPOPLASIA> radial aplasia

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12
Q

What skin anomalies may occur?

A

cafe au lait spots

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13
Q

What hemato problems arise d.t inherited bone marrow failure?

A
  • macrocytosis, FOLLOWED by thrombocytopenia, then neutropenia
  • —risk of APLASIA (84% by 20 years)
  • —-risk of leukaemia (52% by 40 years)
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14
Q

Why may hemato problems arise?

A
  • d.t the inability of the blood cells to CORRECT their inter-strand cross-links (DNA damage)
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15
Q

Name acquired PRIMARY bone marrow failure.

A
  1. idiopathic aplastic anaemia
  2. Myelodysplastic syndromes
  3. Acute Leukaemia
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16
Q

Wht occurs as the pathophysiology of idiopathic APLASTIC anaemia?

Only manifestation?

A
  • multipotent MYELOID stem cells are suppressed d.t T-cell overactivity
  • –only erythroid progenitors are affects

—anaemia is the only manifestion

17
Q

What are some myelodysplastic syndromes?

A
  1. DYSPLASIA (d.t DISORDERED development) !
  2. HYPERCELLULAR marrow
  3. INCREASED APOPTOSIS of progenitor and MATURE cells (ineffective haemopoiesis)
18
Q

What can the MDS evolve into? WHY?

A

AML
—because the abnormal stem cell clone in the marrow is GENETICALLY unstable and PRONE to the acquisition of ADDITIONAL mutations

19
Q

What is the pathophysiology of myelodysplastic syndrome?

A

bone marrow is partly or wholly replaced by CLONAL progeny of multipotent stem cell that CAN differentiate into red cells, platelets and granulocytes
—-BUT in INEFFECTIVE and DISORDERLY way

20
Q

What is seen as a consequence of MDS, in a marrow sample?

A
  • marrow that is HYPERCELLULAR/ normocellular

- but peripheral blood show one or more cytopenias

21
Q

What cells would you expect to see in a case of MDS, in the marrow?

A
  1. megaloblastoid erythroid precursors (resembles megaloblastic anaemia) —ringed sideroblasts
  2. small megakaryocytes with SINGLE/ small nuclei OR multiple seprate nuclei
  3. granuocytes with abnormal granules
22
Q

Why can acute leukaemia cause pancytopenia?

A
  • proliferation of abnormal cells from leukaemic stem cells
  • fails to differentiate/mature into NORMAL cells

—prevent NORMAL stem cell development; hijacking the haemopoietic NICHE
and marrow microenv.

23
Q

What induces bone marrow failure?

A

(IIary)
1. Drugs

  1. B12/ Folate def.
  2. Infiltrative- Non-hemopoietic MALIGNANT infiltration/ lymphoma
  3. others: Viral (HIV)/ storage diseases
24
Q

What drugs cause marrow failure?

A

CHEMO
Chloramphenicol
Alcohol

—known to cause APLASIA

25
Q

What incr.-destruction, results in pancytopenia?

A
  1. Hypersplenism
26
Q

What occurs with hypersplenism?

A
  • incr. splenic pool

- incr. destruction that EXCEEDS bone marrow capacity

27
Q

What causes high rate of sequestration in hypersplenism?

A

those with a red cell mass of 40%

—–mean transit time for red cells will be SLOW !

28
Q

Does splenic size alone, correlate with hypersplenism?

A

NO

—-but splenomegaly CAN potentially result in hypersplenism

29
Q

Causes of HYPERSPLENISM.

A
  1. splenic congestion (portal hypertension, CHF)
  2. Systemic diseases (RA- Felty’s)
  3. Haematological diseases (Splenic lymphoma)
30
Q

Clinical fts of pancytopenia?

A
  • lack of circulating blood cells

- anaemia+ neutropenia+ thrombocytopenia

31
Q

What are the symptoms of anaemia?

A
  1. fatigue
  2. SOB
  3. CVS compromise
32
Q

Thrombocytopenic fts?

A
  • bleeding (purpura/ petechiae/ wet bleeds
33
Q

Ivx for pancytopenia?

A
  1. FBC, blood film
  2. B12/ folate; LFTS; Virology; autoantibody tests)
  3. bone marrow examination
  4. special test (cytogenetics/ chromosome fragility)
34
Q

With suspected Fanconi’s anaemia, what ivx is done?

A
  • cytogenetics
35
Q

When is a hypercellular marrow seen?

A
  • MDS
  • B12/Folate def. (late maturation FAILURE+ Early proliferation+ apoptosis)
  • hypersplenism
36
Q

What supportive treatment can be given for pancytopenia?

A
  • Red cell transfusions
  • platelet transfusions
  • neutrophil transfusions (though NOT routine)
  • antibiotic prophylaxis/ treatments (anti-fungals/ antibacterials)
37
Q

What is a possible rx for hypersplenism?

A

SPLENECTOMY

—–not ALWAYS the case

38
Q

If the pt has a CONGENITAL bone marrow disorder, what should be given as a treatment?

A
  • bone marrow transplantation!
39
Q

How to manage idiopathic aplastic anaemia?

A

by immunosuppression

HEMATOLOGY (20 decks)

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