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Haematology week 2 > Pancytopenia 2 > Flashcards

Pancytopenia 2 Flashcards

1
Q

What is the main cause of pancytopenia due to increased destruction ?

A

Hypersplenism

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2
Q

What is the characterisitic triad of hypersplenism?

A
  1. Splenomegaly (doesnt always correlate to hypersplenism)
  2. Cytopenias with respective bone marrow hyperplasia of precursors, and
  3. Resolution of the cytopenias with splenectomy

Increased splenic pool

Increased destruction that exceeds bone marrow capacity, usually associated with significantly enlarged spleen - it is an condition in which the spleen becomes increasingly active and then rapidly removes the blood cells

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3
Q

What is meant by the splenic pool ?

A

Cells contained within the spleen

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4
Q

Compare the normal splenic pool to the splenic pool in hypersplenism

A

The pancytopenia results from the reduced flow through the liver resulting in increased destruction of blood cells

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5
Q

What are the causes of hypersplenism ?

A

Any cause of splenomegaly can potentially result in hypersplenism

Splenic Congestion:

  • Portal Hypertension
  • Congestive cardiac failure

Systemic diseases:

  • Rheumatoid Arthritis (Felty’s)

Haematological diseases:

  • Splenic lymphoma

Infections:

  • Viral e.g. EBV
  • Syphilis, TB etc
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6
Q

How does hypersplenism often present ?

A
  • Abdo fullness
  • Splenomegaly
  • Early satiety
  • LUQ tenderness
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7
Q

Review the causes of pancytopenia

A
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8
Q

What are some of the symptoms of portal HTN?

A
  • Haematemesis or melaena - suggest bleeding varices.
  • Lethargy, irritability and changes in sleep pattern - suggest encephalopathy.
  • Increased abdominal girth, weight gain - suggest ascites.
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9
Q

What are some of the symptoms of cardiac failure

A
  • Shortness of breath (dyspnea) when you exert yourself or when you lie down (paroxymsmal nocturnal dysponea)
  • Fatigue and weakness
  • Swelling (edema) in your legs, ankles and feet
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10
Q

What are the main signs and symptoms of pancytopenia ?

A
  1. Anaemia
  2. Neutropenia
  3. Thrombocytopenia
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11
Q

What are some of the symptoms of anaemia ?

A
  • Fatigue
  • Shortness of breath
  • Cardiovascular compromise
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12
Q

What are the symptoms resulting from the neutropenia

A

Infections:

  • Severity
  • Duration
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13
Q

What are the symptoms/signs resulting from thrombocytopenia

A

Bleeding:

  • Purpura
  • Petechiae
  • ‘Wet’ bleeds including visceral bleeds
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14
Q

What are the investigations which you do in pancytopenia ?

A
  • History, including family history
  • Clinical findings
  • FBC, Blood film
  • Additional routine tests guided by above (B12/folate, LFT’s, virology, autoantibody tests)
  • Bone marrow examination - aspirate and trephine biopsy
  • Specialised tests guided by above (cytogenetics, eg chromosome fragility testing in Fanconi’s syndrome)
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15
Q

When assessing the bone marrow in pancytopenia what conditions will result in a hypocellular bone marrow and what will result in a hypercellular bone marrow ?

A

Hypocellular in aplastic anaemia

Hypercellular:

  • Myelodysplastic syndromes (proliferation+apoptosis)
  • B12/folate deficiency (late maturation ‘failure’ + early proliferation + apoptosis)
  • Hypersplenism
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16
Q

What is the supportive treatment of pancytopenia ?

A

Bone marrow support:

  • Red cell transfusions
  • Platelet transfusions

Antibiotics treatment and prophylactic use:

  • antibacterials*
  • antifungals
  • Antivirals

Treat neutropenic fever promptly based on local unit antibiotic policy without waiting for (microbiology) results!

17
Q

What is the specific treatment pancytopenia i.e. dependant on the cause ?

A

Primary bone marrow disorder

  • Malignancy – consider chemotherapy
  • Congenital .e.g fanconis anaemia – consider bone marrow transplantation
  • Idiopathic Aplastic Anaemia – only bone marrow support and neutropenic regime, if young and severly affected then allogenic stem cell transplant may cure. Otherwise Immunosuppression with ciclosporin and antithymocyte globulin

Secondary bone marrow disorder:

  • Drug reaction – STOP
  • Viral – eg treat HIV
  • Replace B12/folate

Hypersplenism:

  • Treat cause if possible
  • Consider splenectomy (not appropriate in all cases as you may be able to treat the cause or supportive therapy may be more appropriate)
18
Q

What is the treatment given for neutropenic fever (sepsis)?

A

If NEWS score <6 then at moderate risk and treat with the following:

  • Piperacillin or Tazobactam

If NEWS score >6 then at high risk and treat as follows:

  • (Piperacillin or Tazobactam) + Gentamicin

Haematology week 2 (17 decks)

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