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WILLIAM'S HEMATOLOGY > 57 Hypersplenism and Hyposplenism > Flashcards

57 Hypersplenism and Hyposplenism Flashcards

1
Q

Defined as blood cytopenias in the setting of splenomegaly

A

Hypersplenism

Accompanied by hyperplasia of the affected cell precursors in the marrow

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2
Q

TRUE OR FALSE

The blood cytopenias are generally corrected by relief of portal hypertension.

A

FALSE

The blood cytopenias are not generally corrected by relief of portal hypertension.

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3
Q

The embryonic spleen appears in the __________of gestation as a multiply lobulated condensation of highly vascular mesenchymal cell aggregates interposed in the arterial circulation in the dorsal mesogastrium.

A

First trimester

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4
Q

Genes are essential for spleen formation, and defects in their expression result in hyposplenia or asplenia

A

HOX11 and WT1 genes

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5
Q

The lymphoid compartment, the white pulp, begins its development early in the __________of gestation, when mature T cells, principally ________ lymphocytes, form a continuous layer along the length of the vessels (periarteriolar sheaths).

A

Second trimester

CD4+ lymphocytes

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6
Q

CD8+ cells reside in_______________ and a specialized subset of γδT cells home to the_________

A

Splenic cords

Pulp

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7
Q

Immunoglobulin (Ig) D+ and IgG+ B lymphocytes form localized deposits, the (primary or secondary) lymph follicles.

A

Primary lymph follicles

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8
Q

(Primary or Secondary) follicles arise later in life, after exposure to immunologic stimuli, and have a distinctive structure that includes a germinal center, a mantle zone, and a marginal zone containing IgM+ and IgG+ B lymphocytes.

A

Secondary follicles

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9
Q

The normal adult spleen weighs _______and has a blood flow that is approximately ____% of the cardiac output.

A

135 ± 30 g

5%

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10
Q

The spleen is composed of (3 components)

A
  • White pulp
  • Marginal zone
  • Red pulp
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11
Q

Approximately__________ of platelets are normally sequestered in the spleen

A

One-third

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12
Q

TRUE OR FALSE

The red pulp plays a major role in adaptive immunity.

A

FALSE

The white pulp plays a major role in adaptive immunity.

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13
Q

The spleen is involved in the phagocytosis of encapsulated bacteria, including:

A
  • Streptococcus pneumoniae
  • Haemophilus influenzae
  • Neisseria meningitidis
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14
Q

The benefits of splenectomy in immune thrombocytopenia is a result of :

A
  • Decreased production of antiplatelet antibodies
  • Decreased clearance by macrophages of antibody-coated platelets through the Fc recognition function of its large macrophage population
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15
Q

Spleen enlargement may result from:

A
  • Expansion of the red pulp compartment with increased blood flow
  • Extramedullary hematopoiesis, notable in primary myelofibrosis
  • Hyperplasia or neoplasia involving the white pulp, such as in infectious mononucleosis or lymphoma; or histiophagocytic hyperplasia
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16
Q

The increased size of the filtering bed is more pronounced when the splenomegaly is caused by congestion as in _____________ than when it is caused by cellular infiltration as in leukemias, extramedullary hematopoiesis, or amyloidosis.

A

Portal hypertension

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17
Q

TRUE OR FALSE

Sequestered white cells and platelets survive in the spleen and may be available when increased demand requires neutrophils or platelets, although their release may be slow.

A

TRUE

Sequestered white cells and platelets survive in the spleen and may be available when increased demand requires neutrophils or platelets, although their release may be slow.

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18
Q

Causes of Massive Splenomegaly

A
  1. Myeloproliferative disorders
    a. Primary myelofibrosis
    b. Chronic myeloid leukemia
  2. Lymphomas
    a. Hairy cell leukemia
    b. Chronic lymphocytic leukemia (especially
    prolymphocytic variant)
  3. Infectious
    a. Malaria
    b. Leishmaniasis (kala azar)
  4. Extramedullary hematopoiesis
    a. Thalassemia major
  5. Infiltrative
    a. Gaucher disease
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19
Q

TRUE OR FALSE

Slight to moderate enlargement of the spleen usually does not produce local symptoms.

A

TRUE

Slight to moderate enlargement of the spleen usually does not produce local symptoms.

Even massive splenomegaly can be well tolerated if it develops gradually.

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20
Q

Splenic rupture is uncommon but can occur spontaneously with most causes of:

A

Splenic enlargement or after blunt trauma

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21
Q

TRUE OR FALSE

Generally, a palpable spleen signifies splenomegaly and is measured by the number of centimeters the spleen extends below the left costal margin.

A

TRUE

Generally, a palpable spleen signifies splenomegaly and is measured by the number of centimeters the spleen extends below the left costal margin.

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22
Q

Splenic size is most accurately measured with

A

Abdominal ultrasound or computed tomographic scans

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23
Q

Imaging used primarily to identify cysts, abscesses, and infarcts

A

Magnetic resonance imaging

24
Q

A wandering spleen

An uncommon phenomenon in which the spleen hangs by a long pedicle of mesentery

A

Splenoptosis

25
Q

Splenoptosis may present in three ways:

A

(a) an asymptomatic mass in the pelvis;
(b) intermittent abdominal pain with or without gastrointestinal symptoms; or less often,
(c) an acute abdomen resulting from torsion

Initially mistaken for a pelvic or lower abdominal tumor

26
Q

The characteristic features of hypersplenism are

A
  • Splenomegaly
  • Blood cytopenias
  • Absence of other causes of cytopenias (eg, anemia caused by bleeding)
27
Q

Thrombocytopenia is a common finding in patients with:

A

Hepatic cirrhosis, portal hypertension, and splenomegaly

28
Q

TRUE OR FALSE

The presence of thrombocytopenia or leukopenia in patients with chronic liver disease is associated with increased mortality.

A

TRUE

The presence of thrombocytopenia or leukopenia in patients with chronic liver disease is associated with increased mortality.

Decompensated liver disease and history of alcohol consumption are independent risk factors for hypersplenism, but why some patients develop marked blood cytopenias is not clear, although folate deficiency is a factor in some instances.

29
Q

TRUE OR FALSE

The response to transfusion of blood products, especially platelets, is not affected in patients with massive splenomegaly.

A

FALSE

The response to transfusion of blood products, especially platelets, may be significantly impaired in patients with massive splenomegaly.

30
Q

Indications for total spenectomy

A
  • Abdominal trauma and partial rupture of the spleen
  • Splenic size or infarcts causes sustained left upper abdominal pain or discomfort
  • Treatment of functionally significant blood cytopenias
31
Q

The most common indications for splenectomy

A
  • Hereditary spherocytosis
  • Immune thrombocytopenia
  • Immune hemolytic anemia

Others: thalassemia major, sickle cell anemia

Improving cell survival and also by decreasing autoantibody production.

32
Q

TRUE OR FALSE

Splenectomy in patients with a massive spleen size (>1500 g), especially in CML, is accompanied by higher morbidity and mortality than is removal of the spleen for immune blood cytopenia.

A

FALSE

Splenectomy in patients with a massive spleen size (>1500 g), especially in primary myelofibrosis, is accompanied by higher morbidity and mortality than is removal of the spleen for immune blood cytopenia.

33
Q

An advantage of open splenectomy in hematologic conditions such as the treatment of immune thrombocytopenia is

A

Ease of searching assiduously for accessory spleens

34
Q

Systemic complications after splenectomy

A
  • Infections from encapsulated organisms
  • Venous and arterial thromboembolic events
35
Q

Modes of doing partial splenectomy

A
  • Ligation of some of the splenic arteries
  • Arterial embolization
36
Q

It can be used in patients with an absolute contraindication to splenectomy who might benefit symptomatically from reduction of a massively enlarged spleen

A

Splenic Radiation

37
Q

Splenic radiation may be associated with severe cytopenias and especially thrombocytopenia termed as

A

Abscopal effect

38
Q

TRUE OR FALSE

Thrombocytopenia may not be corrected after liver transplant if the splenomegaly persists.

A

TRUE

Thrombocytopenia may not be corrected after liver transplant if the splenomegaly persists.

39
Q

Thrombopoietin Receptor Agonists have studies that it increase platelets in patients with thrombocytopenia as a result of

A

Hepatitis C virus–related cirrhosis

40
Q

The designation for decreased splenic function resulting from:
* Diseases that impair function
* Absence of splenic tissue because of agenesis, atrophy (eg, autoinfarction of sickle cell disease), or splenectomy

A

Hyposplenism

41
Q

TRUE OR FALSE

Splenic hypofunction may be associated with a normal spleen size.

A

TRUE

Splenic hypofunction may be associated with a normal spleen size.

42
Q

Infectious disease that cause hyposplenism

A
  • Malaria
  • Disseminated meningococcemia
43
Q

PBS findings in hyposplenism

A

Howell-Jolly bodies and erythrocyte pits

44
Q

The most common causes of hyposplenism

A
  • Sickle cell anemia
  • Surgical splenectomy
45
Q

TRUE OR FALSE

Although the presence of an enlarged spleen usually suggests hypersplenism, spleen size is not a reliable index of splenic function.

A

TRUE

Although the presence of an enlarged spleen usually suggests hypersplenism, spleen size is not a reliable index of splenic function.

46
Q

Example of hyposplenic splenomegaly

A

Complete splenic replacement by cysts, neoplastic tissue, or amyloid

47
Q

Congenital asplenia may be found in infants with _________ and other developmental abnormalities.

A

Situs inversus

48
Q

TRUE OR FALSE

Splenic replacement by neoplastic cells, as in lymphomas and leukemias, usually does not cause hyper- or hyposplenism.

A

TRUE

Splenic replacement by neoplastic cells, as in lymphomas and leukemias, usually does not cause hyper- or hyposplenism.

Hematologic

49
Q

Splenectomy in children should be deferred until _____years of age.

A

5 years of age

50
Q

The risk of overwhelming sepsis post splenectomy is reduced by the use of

A

Pneumococcal and H. influenzae vaccines before splenectomy and prophylactic penicillin therapy

51
Q

The most specific of all the blood findings

A

Pitted erythrocytes in wet preparations

Other blood findings: Howell-Jolly bodies, target cells, Pappenheimer (siderotic) bodies, and occasional acanthocytes

Followed by the presence of DNA inclusions in circulating red cells (Howell-Jolly bodies)

Target cells reflecting an increased red cell surface are almost always present in the asplenic state, but only 1 in 100 to 1 in 1000 red cells is affected

52
Q

A sensitive indication of hyposplenism

A

Pits or pocks on the cell surface

53
Q

Pits or pocks on the cell surface consist of

A

Submembranous vacuoles

Can be seen only in wet preparations of red cells using direct interference-contrast microscopy

54
Q

A reliable measure of the capacity of the spleen to clear particulate matter from the bloodstream

A

Spleen scanning with Technetium-99m sulfur-colloid particle

55
Q

Antibiotic prophylaxis for asplenic patients that is recommended based on publicized guidelines

A

Oral penicillin or a macrolide antibiotic

56
Q

TRUE OR FALSE

All asplenic patients with any febrile episode (>38°C) should be considered an emergency requiring immediate medical attention.

A

TRUE

All asplenic patients with any febrile episode (>38°C) should be considered an emergency requiring immediate medical attention.

WILLIAM'S HEMATOLOGY (109 decks)

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