Hematologic pathology Flashcards

1
Q

What is the difference between Primary & secondary polycythemia??

A
  1. Primary: polycythemia vera or erythremia, is a myeloproliferative syndrome. Unknown cause
    * There is increase in RBC/WBC/Platelets count
    * Erythropoietin level is low
    * Later, anemia or acute leukemia due to bone marrow burnout
  2. Secondary: Increase RBC mass due to high erythropoietin. Due to high altitude, low O2, smoking, right to left shunt, renal disease (including malignancy)
    * No increase in platelets/WBC count
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2
Q

What is the difference between relative and absolute polycythemia??

A
  1. Relative: due to loss of intravascular volume with no loss of RBC count
  2. Absolute: Increase in RBC count
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3
Q

Normal platelet count is ……

A

150k - 400k / mm3

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4
Q

What are the most common signs of thrombocytopenia?

A
  1. Petechia: minute, pin sized hemorrhage in skin

2. Purpura: large, red, non blanching lesions

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5
Q

In splenomegaly, platelets breakdown is ……

A

increased

* Leads to thrombocytopenia

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6
Q

Thrombotic thrombocytopenic purpura is ……..

A

Formation of small thrombi throughout the body, leading to organ damage and thrombocytopenia

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7
Q

Idiopathic thrombocytopenic purpura is …….

A

low platelets, with normal bone marrow. Purpura is formed with no trauma, bleeding from the nose, CNS, GIT, hematuria

  • Could be acute (following viral infection), or chronic
  • Could be secondary to hemolytic anemia, SLE, HIV
  • There is splenic destruction of opsonized platelets
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8
Q

Platelet function defect affects the ………

A

bleeding time

* In the presence of normal platelet count

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9
Q

Examples of defect in platelet adhesion are ….

A

von Willbrand disease, Bernard soulier syndrome

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10
Q

Von Willbrand disease is …….

A

autosomal dominant defect in von Willbrand factor.

* Factor VIII is also affected

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11
Q

Thrombocytosis normally don’t cause thrombosis because ……..

A

in myeloproliferative disorders, the platelets do not function normally

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12
Q

Thrombocythemia is an essential feature of …….

A

chronic myelogenous leukemia (CML)

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13
Q

Disorders of primary hemostasis are ….

A

caused by defects in platelets and vessels

* Occurs later in life. Usually bleeding in superficial vessels (petechia)

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14
Q

Disorders of secondary hemostasis are ……

A

due to problems with clotting factors

  • Bleeding occurs in deep vessels
  • PTT increase (internal), PT increased (external), BT normal
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15
Q

Thrombin time is used to measure …….

A

Factor I (fibrinogen)

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16
Q

Vit K is needed for ……..

A

post translational modification of factors II, VII, IX & X, as well as factor C & S
* Warfarin causes Vit K deficiency

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17
Q

What clotting factors are synthesized in the liver??

A

Factors II, V, VII, IX, X, XI & XII

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18
Q

DIC is caused by ……..

A

activation of clotting system, with subsequent consumption of clotting factors, platelets & fibrin. This lead to activation of fibrinolytic system
* Death may occur due to thrombosis or hemorrhage

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19
Q

Factor VIII is synthesized in ……..

A

endothelium of vessels

* The rest of clotting factors are synthesized in the liver

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20
Q

Coagulopathy with low factor VIII suggests ……

A

DIC

* In liver coagulopathy, factor VIII level is normal

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21
Q

Neutropenia is seen in patients treated with ……

A

alkylating agents

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22
Q

Neutropenia causes high susceptibility to infection, especially ……

A

G -ve septicemia

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23
Q

Eosinophilic leukocytosis is seen in …..

A

neoplasms, allergy, asthma, collagen vascular disease, parastitc infections
* Any skin rash may produce eosinophilia

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24
Q

Left shift is ……

A

an increase in the number of immature leukocytes in the peripheral blood, particularly neutrophils

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25
Q

Chronic myelogenous leukemia produces …..

A

extreme leukocytosis with immature eosinophils and basophila

26
Q

Leukocyte alkaline phosphatase is increased in ……., and decreased in …….

A
leukomoid reaction (due to stress or infection)
Acute and chronic myelogenous leukemia
27
Q

Generalized lymphadenopathy may be a precursor for ……..

A

AIDS

28
Q

Hodgkins lymphoma spread in …….

A

contiguity (from one set of lymph nodes to the next)

* Spleen is affected before the liver

29
Q

Hodgkins lymphoma causes leukemia. T/F??

A

False

  • There is no leukemic component
  • There is high cure rate
30
Q

Reed Sternberg cells are …….

A

characteristic of Hodgkins lymphoma

  • Large (15-40 micrometer), with two or more nuclei containing “owl-eyed” eosinophilic nucleoli
  • Cytoplasm is abundant
  • Not enough to confirm diagnosis
31
Q

The clinical features of Hodgkins lymphoma are …….

A

Painless cervical adenopathy, with fever, chills, night sweats and weight loss

32
Q

The prognosis of Hodgkins lymphoma depends on ……….

A

the age & stage

33
Q

Non Hodgkins lymphoma differs from Hodgkins lymphoma by …….

A
  1. No Reed-Sternberg cells
  2. Doesn’t spread in contiguity
  3. Have a leukemic or blood-borne phase
    * Common in immunosuppressed patients
34
Q

What are the types of non Hodgkins lymphoma??

A
  1. Well differentiated leukocyte
  2. Poorly differentiated leukocytes
  3. Histiocytic lymphoma
  4. Lymphoblastic lymphoma
  5. Burkitt (undifferentiated lymphoma)
35
Q

Acute leukemia is characterized by ……

The most common types are ……… & ……..

A

presence of blasts in the peripheral blood and lack of mature cells

  • Acute lymphocytic and acute myelogenous
  • Acute monocytic and acute undifferentiated are less common
  • More in children & over 60
36
Q

Chronic leukemia is characterized by ……..

The most common forms are ….. & ……

A

increased number of mature WBCs in the peripheral blood

  • chronic lymphocytic and chronic myelogenous are the most common
  • Chronic monocytic is less common
37
Q

Generally, leukemia is characterized by ……….

A

uncontrolled proliferation of abnormal monoclonal cells, with increase number of blast cells in the blood and marrow.
* There is decrease in RBC, WBC & platelets

38
Q

The most common leukemia in children is …..

A

acute lymphocytic leukemia

  • There is anemia & thrombocytopenia, lymphadenopathy & hepatosplenomegaly, bone pain
  • Mature WBC are rare
  • Good prognosis
39
Q

Acute myelogenous leukemia is …….

A

common in adults, poor prognosis

  • Same signs as ALL
  • Large Myeloblasts with round nucleus & two or more nucleoli, basophilic cytoplasm
  • Contain Auer Rods (fused lysosomes)
40
Q

Chronic leukemia is characterized by ……..

A

Higher number of differentiated WBC, less severe than acute leukemia and takes longer time. Symptoms are the same
* Weight loss is common

41
Q

Blast crisis may develop in patients with …….

A

chronic myelogenous leukemia and lead to death

* It is an acute form of leukemia with high number of blasts in blood

42
Q

The cause of CML is ………

A

translocation between chromosome 9 & 22 (Philadelphia chromosome)

43
Q

CML is characterized by ………

A

high number of WBC (however, lymphocytes are low)

* The spleen is massively enlarged

44
Q

Chronic lymphocytic leukemia is …..

Characterized by ……..

A

the most common type of adults leukemia and the least malignant.

  • Proliferation of abnormal B lymphcytes (can not produce AB), leading to more bacterial infections
  • Normochromic normocytic anemia is common. Autoimmune hemolysis may occur
  • Also called lymphocytic lymphoma
45
Q

Adult T-Cell leukemia/lymphoma is caused by ……

Characterized by ……..

A

HTLV 1, a virus endemic in Japan, similar to HIV

  • Lymphadenopathy, hepatosplenomegaly, hypercalcemia
  • Incubation period may be decades
  • The primary cell type is CD4 T cell
46
Q

Promyelocytic leukemia is a subtype of ……..

A

AML

47
Q

Hypergammaglobulinemia is …….

A

increased serum level of Ig

48
Q

What are the types of hyperimmunoglobulinemia?

A
  1. Monoclonal: increase in one type. There is increased of specific circulating Ig and also fragment of immunoglobin (found in urine). May be malignant (M myeloma, Waldenstrom, heavy chain disease) or benign
  2. Polyclonal: Usually 1-2 weeks after antigenic stimulation. Liver disease increase immunoglobulins due to decreased catabolism
49
Q

What are the Lab signs for hypergammaglobulinemia?

A

High immunoglobulins, high ESR, positive serum protein electrophoresis, free light chain (Bence-Jones protein) in serum or urine

50
Q

In multiple myeloma, the free light chains are ……., while in liver disease, inflammation, and glomerulopathy it is …..

A

monoclonal

polyclonal

51
Q

What are the clinical features of hypergammaglobulinemia??

A
  1. Hyperviscosity of blood, leads to thrombosis, hemorrhage, renal & CNS impairment, right heart failure
  2. Cryoglobulins (esp. M component) percipitatation of immunoglobuins in cold, causing Raynaud’s phenomenon, thrombosis & gangrene
  3. High immunoglobulins with AB may lead to RBC, granulocytes, platelets destruction
52
Q

Multiple myeloma is …….., causes …….., features ……..

A

plasma cell neoplasm in bone marrow

  • Overproduction of monoclonal IgG
  • hyperviscosity, lack of functioning AB leads to infections, proteinuria (due to plugging by casts of myeloma protein, amyloid), bone pain & hypercalcemia
    • Bence Jones protein is elevated in serum or urine
  • Marrow shows punch-out radiolucencies on Xray
53
Q

Waldenstrom macroglobulinemia is …….

A

neoplasm of plasma cells that produce IgM

  • There is no lytic bone lesions (like in m myeloma)
  • Same symptoms as m myeloma
  • Bence Jones protein is present in urine
54
Q

Hypersplenism is ……..

A

increased activity by enlarged spleen. Causes sequestration of blood elements
* Characterized by splenomegaly, anemia, leukopenia or thrombocytopenia

55
Q

What are the causes for splenomegaly??

A
  1. Infections: CMV, TB, malaria, mononucleosis
  2. Portal hypertension: cirrhosis, right HF, portal vein thrombosis
  3. Inflammation: SLE, RA
  4. Lymphohemogenous disease (myeloma, lymphoma, leukemia
  5. Storage disease: Gaucher, Neimann Pick
  6. Amyloid, infarcts, tumors
56
Q

Splenic infarcts initially present with ……

It is caused by …….

A

splenomegaly, then fibrosis and shrinkage occurs

* occlusion of splenic artery, embolism from heart. Occlusion by sickled cells produces multiple infarcts

57
Q

What are the causes of thymus atrophy??

A

Congenital, radiation, chemotherapy, stress

58
Q

Thymoma is …..

A

tumor of the thymus, originating from its epithelium (thymus cover)

  • Mostly benign
  • Associated with Myasthenia gravis
59
Q

Thymic lymphoma is ……..

may be associated with …….

A

Tumor of lymphocytes in the thymus

* Hodgkins & non Hodgkins, and ALL

60
Q

To diagnose DIC, there should be ……..

A

hypofibrinogenmia, thrombocytopenia, fibrin degradation products, and a prolonged PT/PTT/BT