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WILLIAM'S HEMATOLOGY > 3 Consultative Hematology > Flashcards

3 Consultative Hematology Flashcards

1
Q

The most common type of anemia in outpatients

A

Iron-deficiency anemia

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2
Q

The most common type of anemia in hospitalized patients

A

Anemia of chronic inflammation

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3
Q

The most common source of occult blood loss in

Men:
Women:

A

Men: gastrointestinal tract

Women: blood loss secondary to menorrhagia

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4
Q

Evaluation of anemia

Of the RBC indices, the ______ is most useful.

A

MCV

  • Microcytic (MCV < 80 fL)
  • Normocytic (MCV 80–100 fL)
  • Macrocytic (MCV > 100 fL)
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5
Q

Useful in evaluating whether anemia is from decreased RBC production by the marrow versus increased destruction

A

Reticulocyte index

  • Low reticulocyte index [< 2.0]
  • High reticulocyte index [≥ 2.0]
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6
Q

TRUE OR FALSE

Red cell size is best assessed from the MCV because the blood film is a 2-dimensional assessment of a 3-dimensional question (volume).

A

TRUE

Red cell size is best assessed from the MCV because the blood film is a 2-dimensional assessment of a 3-dimensional question (volume).

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7
Q

Causes of microcytic anemia

A
  • Iron deficiency
  • Thalassemia minor
  • Sideroblastic anemia (hereditary, lead poisoning)
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8
Q

Causes of normocytic anemia

A
  • Anemia of chronic inflammation
  • Anemia of chronic kidney disease
  • Primary marrow disorders
  • Hemolytic anemias
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9
Q

Toxin causing normocytic anemia, basophilic stippling of the erythrocytes may be fine or course with a variable number of granules in each cell

A

Lead poisoning

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10
Q

Causes of macrocytic anemia

A
  • Deficiencies of folate and vitamin B12
  • Acute megaloblastic anemia
  • Drug induced
  • Alcohol abuse
  • Inborn errors of metabolism
  • Clonal hematologic disorders including myelodysplastic syndromes
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11
Q

Neutropenia refers to values less than 2 SD below the normal mean of the population, and corresponds to

Children 1-10yo:
Above 10 yo:

A

Children 1-10: less than 1.5 × 109/L
Above 10: less than 1.8 × 109/L

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12
Q

An inherited cause of neutropenia in individuals of African descent and certain other ethnic groups in which the mean and 95% confidence interval of ANC is significantly lower than that of persons of European descent

A

Benign ethnic neutropenia

Neutropenia in persons of European descent may be mild (ANC <1.8 × 109/L), moderate (ANC <1.0 × 109/L), or severe (ANC <0.5 × 109/L).

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13
Q

Causes of chronic neutropenia

A
  • (a) congenital neutropenia (neutropenia present at or near birth secondary to a marrow failure syndrome primarily involving the granulocytic series);
  • (b) cyclic neutropenia (a rare autosomal-dominant genetic disorder characterized by neutropenia that recurs every 14 to 35 days);
  • (c) chronic idiopathic neutropenia (no obvious cause for the neutropenia; ANC ranges from 0.5 to 1.0 × 109/L);
  • (d) chronic infections (particularly hepatitis and HIV);
  • (e) nutritional deficiencies (vitamin B12, folate, or copper); and
  • (f) autoimmune disorders

With respect to autoimmune disorders, nearly half of patients with systemic lupus erythematosus (SLE) have neutropenia, often with accompanying monocytopenia and lymphopenia.

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14
Q

Thrombocytopenia is defined in adults as being below the lower limit of normal (150 × 109/L),

Mild:
Moderate :
Severe:

A
  • Mild: (70–150 × 109/L)
  • Moderate : (20–70 × 109/L)
  • Severe: (< 20 × 109/L)
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15
Q

Causes of thrombocytopenia

A
  • Impaired platelet production
  • Increased platelet destruction
  • Abnormal distribution
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16
Q

A finding of more than 10% megathrombocytes (platelets greater in diameter than one-third of a red cell diameter) suggests

A

Immune platelet destruction

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17
Q

It usually occurs 5–10 days after exposure to heparin and is associated with both venous and arterial thrombosis.

The thrombocytopenia can be mild, and bleeding is unusual.

A

HIT

The specifics of diagnostic tests, including an immunoassay for antiplatelet factor-4

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18
Q

In a patient with neurologic symptoms, microangiopathic hemolysis, and an elevated serum creatinine, ____________ should be ordered in addition to instituting prompt plasmapheresis for the tentative diagnosis of TTP.

A

A disintegrin and metalloprotease with a thrombospondin type 1 motif member 13 (ADAMTS-13) activity level

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19
Q

Causes of thrombocytopenia in pregnancy

A
  • Preeclampsia
  • HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome
  • Abruptio placentae with DIC
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20
Q

Acquired and Congenital Causes of Pancytopenia

A

Acquired
* Marrow infiltration/replacement
* Marrow aplasia
* Blood cell destruction/sequestration/redistribution
* Combination of causes

Congenital
* Wiskott-Aldrich syndrome
* Fanconi anemia, dyskeratosis congenita
* Shwachman-Diamond syndrome
* GATA2 deficiency
* Hemophagocytic lymphohistiocytosis

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21
Q

Refers to an increase in hemoglobin concentration

A

Erythrocytosis

Relative erythrocytosis: erythrocytosis may be caused by a decrease in plasma volume

Absolute erythrocytosis: an increase in the RBC mass

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22
Q

The most common reasons for a decrease in plasma volume

A

Dehydration from vomiting, diarrhea, or diuretic use

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23
Q

Causes of absolute erythrocytosis

A
  • Acquired or hereditary autonomous production of RBCs (primary erythrocytosis)
  • Acquired or hereditary appropriate physiologic response to an increased production of serum erythropoietin (EPO) (secondary erythrocytosis)
  • Disorders of hypoxia signaling
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24
Q

Myeloid neoplasm in which the increased red cell mass frequently accompanies an increase in granulocytes and platelets

Associated with Janus kinase 2 (JAK2) mutations

A

PV

The findings of splenomegaly, thrombocytosis, and/or granulocytosis are highly suggestive of PV and should trigger mutational testing for JAK2V617F (exon 14), and if the findings are negative, JAK2 exon 12 mutation.

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25
Q

Most common causes of secondary erythrocytosis

A

(a) cardiopulmonary states that lead to hypoxemia,
(b) carboxyhemoglobinemia resulting from smoking or toxic exposure,
(c) autonomous EPO production from malignancies or polycystic kidney disease, and
(d) exogenous EPO administration (“blood doping”)

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26
Q

Neutrophilia refers to an increase in the band and segmented neutrophils to a combined concentration greater than 2 SD above the normal population mean value, or approximately__________ × 109/L

A

7.5 × 109/L

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27
Q

Most common cause of neutrophilia

A

Inflammatory and infectious diseases

Other secondary causes of neutrophilia include smoking, obesity, stress (both physical and emotional), asplenia, and medications

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28
Q

Primary marrow disorders associated with neutrophilia

A
  • Chronic myelogenous leukemia (CML)
  • Other myeloproliferative neoplasms
  • Chronic neutrophilic leukemia (CNL)
  • Sickle cell disease

In a patient with persistent neutrophilia of unclear etiology, it is generally advisable to exclude CML with a qualitative breakpoint cluster region-Abelson (BCR-ABL) polymerase chain reaction (PCR) analysis.

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29
Q

CNL, a rare disorder can be considered when the WBC must be greater than ________ with greater than 80% neutrophils

Splenomegaly is a very frequent feature

A

25 × 109/L

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30
Q

Genetic testing for the_________ gene mutation, present in approximately 90% of cases, is confirmatory of CNL

A

CSF3R

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31
Q

Medications that cause neutrophilia

A
  • Glucocorticoids
  • Lithium
  • Exogenous growth factors such as granulocyte colony-stimulating factor
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32
Q

Eosinophilia

Mild
Moderate
High

A

Eosinophilia
* Mild (< 1.0 × 109/L)
* Moderate (1.0–5.0 × 109/L)
* High (> 5.0 × 109/L)

The normal absolute eosinophil count is less than 0.4 × 109/L.

33
Q

The most common cause of eosinophilia

A

Infection with helminthic parasites

In industrialized countries, the allergic diseases (allergic rhinitis, atopic dermatitis, and asthma) are the most common causes with mild to moderate degrees of eosinophilia.

34
Q

Rhinosinusitis, asthma, and eosinophilia require screening for

A

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

35
Q

Basophils are the least common of the circulating WBCs, and typically account for less than ___________ % of blood leukocytes.

A

Less than 0.5–1%

Basophilia may be associated with:
* Allergic or inflammatory responses
* Endocrinopathies (diabetes, myxedema)
* Infections (chicken pox, influenza, smallpox, tuberculosis)
* Iron deficiency
* Clonal myeloid diseases (especially CML, but also PV, primary myelofibrosis, ET, acute myelogenous leukemia [AML], and basophilic leukemias)

36
Q

TRUE OR FALSE

If the medical history, physical examination, or laboratory findings do not support other potential etiologies, CML should be considered as virtually all patients have basophilia.

A

TRUE

If the medical history, physical examination, or laboratory findings do not support other potential etiologies, CML should be considered as virtually all patients have basophilia.

37
Q

The usual presenting skin lesion in mastocytosis appearing as small yellowish tan to reddish-brown macules or papules

A

Urticaria pigmentosa

38
Q

Monocytosis in adults is present when the absolute count exceeds_____ × 109/L

A

0.8 × 109/L

The causes of monocytosis include:
* Myeloid and lymphoid neoplasms
* Chronic and drug-induced neutropenia
* Immune hemolytic anemia
* A variety of inflammatory and immune disorders
* Infections
* Gastrointestinal disorders
* Following a myocardial infarction
* Postsplenectomy

39
Q

Unexplained monocytosis, particularly in elderly patients with other cytopenias, should raise concern for myeloid malignancies, such as__________________ , and generally warrants examination of the marrow.

A

MDS and chronic myelomonocytic leukemia (CMML)

40
Q

In adults, lymphocytosis refers to an ALC that exceeds ________× 109/L

A

4 × 109/L

*The normal absolute lymphocyte count (ALC) is higher in children than adults. *

41
Q

Primary lymphocytosis disorders include:

A
  • Acute and chronic lymphocytic leukemia
  • Prolymphocytic leukemia
  • Hairy cell leukemia
  • The leukemic phase of B-cell lymphoma
  • Large granular lymphocytic leukemias (LGLLs)
42
Q

Reactive lymphocytosis is most commonly caused by

A
  • Infectious mononucleosis (presenting symptoms may include fever and tonsillary pharyngitis with cervical lymphadenopathy)
  • Bordetella pertussis
  • Natural killer (NK) cell lymphocytosis
  • Stress (cardiovascular collapse, toxic shock, trauma, major surgery)
  • Hypersensitivity reactions to insects or drugs
  • Subacute or chronic persistent lymphocytosis from conditions such as smoking, hyposplenism, chronic infections, or thymoma
43
Q

There are 4 main causes of thrombocytosis:

A
  • (a) clonal
  • (b) reactive (secondary)
  • (c) familial
  • (d) spurious
44
Q

Causes of spurious thrombocytosis

A
  • Mixed cryoglobulinemia
  • Fragmented red blood cells
  • Bacteria
45
Q

Familial thrombocytosis caused by mutations in the gene for the thrombopoietin receptor, ______, the thrombopoietin gene ______

A

Thrombopoietin receptor: MPL
Thrombopoietin gene : THPO

46
Q

TRUE OR FALSE

Blast cells in the blood are nearly always indicative of a hematopoietic neoplasm.

A

TRUE

Blast cells in the blood are nearly always indicative of a hematopoietic neoplasm.

In inflammatory reactions, promyelocytes are rare and myelocytes infrequent.

47
Q

Causes for the appearance of nRBCs (normoblasts) in the blood

A

(a) hypoxia from various cardiopulmonary causes,
(b) stress erythropoiesis (eg, severe hemolytic anemia), and
(c) a myelophthisic condition

A leukoerythroblastic blood film (nRBCs, teardrop RBCs, myeloid immaturity, giant platelets) is a significant clue of marrow infiltration and extramedullary hematopoiesis.

48
Q

The most frequent indications for referral of lymphadenopathy

A
  • Nodes larger than 2 cm
  • The presence of enlarged supraclavicular nodes (up to 50% risk of malignancy in some series),
  • When there are no signs of resolution within a few weeks
49
Q

LADs: Remains the gold standard, but multiple wide diameter core biopsies are a reasonable alternative

A

Excision biopsy

** Multiple wide diameter core** biopsies are a reasonable alternative.

Narrow core biopsies and fine-needle aspirations should be discouraged if there is the likelihood of a lymphoma.

50
Q

Causes of massive splenomegaly

A
  • Myeloproliferative disorders (primary myelofibrosis, CML)
  • Leukemias (hairy cell and CLL)
  • Infections (malaria, leishmaniasis)
  • Thalassemia major
  • Gaucher disease
51
Q

Monoclonal gammopathy

Immunoglobulin G (IgG) and IgA:
Immunoglobulin M:

A

Immunoglobulin G (IgG) and IgA: myeloma, amyloidosis, or lymphoma

Immunoglobulin M: lymphoma or Waldenström macroglobulinemia

52
Q

Patients with a monoclonal immunoglobulin or light chain in the absence of a malignancy have

A

Essential monoclonal gammopathy

53
Q

Diseases associated with a polyclonal gammopathy

A
  • Liver disease (cirrhosis, autoimmune or viral hepatitis)
  • Connective tissue disorders
  • Infections (bacterial, osteomyelitis, endocarditis, viral including HIV)
  • Hematologic disorders (non-Hodgkin lymphoma, chronic CLL, thalassemia, sickle cell disease)
  • Nonhematologic malignancies (lung, ovarian, gastric, hepatocellular)
54
Q

Describes nonblanchable, hemorrhagic skin or mucosal lesions that are a result of bleeding under the surface of the skin

A

Petechiae (< 2 mm)
Ecchymoses (≥ 2 mm)

55
Q

An acute life-threatening disorder resulting from microvascular thrombosis and hemorrhagic infarction

A result of severe disseminated intravascular coagulation (eg, as a complication of meningococcal or streptococcal sepsis)
A consequence of congenital protein C or S deficiency.

Painful lesions are distinguishable by central areas of blue-black hemorrhagic necrosis with a surrounding erythematous border

A

Purpura fulminans

56
Q

The most common cause of vasculitis in children, with the purpura tending to be on the buttocks and lower extremities, plus other clinical signs of arthritis, abdominal pain, and nephritis

A

Henoch-Schönlein purpura

57
Q

The most common cause of acquired platelet dysfunction

A

Aspirin

58
Q

The most common hereditary causes of VTE

A
  • Factor V Leiden (FVL)
  • Prothrombin 20210 mutation
59
Q

Spontaneous upper-extremity VTE

A

Thoracic outlet syndrome

60
Q

Unprovoked left iliofemoral DVT, particularly in women between 20 and 50 years of age

A

May-Thurner syndrome

61
Q

If a D-dimer assay is positive or unavailable, compression ultrasonography with Doppler should be performed, and if negative, repeated in _____ days.

A

5–7 days

62
Q

A PE occurs in up to ________of patients with a ________________ vein thrombosis.

In contrast, a clinically important PE is rarely (1% or less) associated with a thrombosis confined to the calf veins.

A

50%

Proximal vein thrombosis

63
Q

Used to establish or exclude the diagnosis of PE with high predictive value of PE

A

Computerized tomographic angiography (CTA) or combined CTA-computed tomographic venography

64
Q

Characterized by venous or arterial thrombosis or pregnancy complications attributed to placental insufficiency in the presence of persistent laboratory evidence of antiphospholipid antibodies (aPLs)

A

APS

The presence of aPLs alone is insufficient for the diagnosis

65
Q

in APS, 50% of patients will have a DVT of the ________________

A

DVT of the lower extremity

25% of patients present with an arterial thrombosis

25% both venous and arterial thromboses

66
Q

An infrequent presentation of APS characterized by severe, widespread vascular occlusions in at least 3 organs, systems, and/or tissues; development of manifestations simultaneously or in less than 1 week; histopathologic confirmation of small-vessel occlusion; and laboratory evidence of the presence of aPLs

A

Catastrophic antiphospholipid syndrome (CAPS)

67
Q

The laboratory diagnosis of APS requires the demonstration of aPLs and/or antibodies against relevant protein cofactors by immunoassays or coagulation tests performed__________weeks apart

A

12 or more weeks

68
Q

Evaluation for a hereditary cause of thrombophilia is warranted in select populations, including:

A

(a) patients with at least 1 first-degree relative with documented VTE before the age of 45 years

(b) patients without a family history who are younger than 45 years of age, have recurrent thrombosis or thrombosis in multiple sites, or a history of warfarin-induced skin necrosis

69
Q

A febrile illness with anemia, thrombocytopenia, and multiorgan involvement (including splenomegaly, hepatitis, coagulation abnormalities, mental status changes).

A very high serum ferritin ranging from 500 to 10,000 mg/mL

A

Hemophagocytic lymphohistiocytosis (HLH)

Infections, most commonly an Epstein-Barr viral infection, can disrupt immune homeostasis and lead to this aggressive and life-threatening syndrome.

70
Q

The most useful test to discern iron overload from other causes

A

Transferrin saturation (TSAT)

Ratio of serum iron to the total iron-binding capacity (TIBC)

TSAT, 45% or greater in men and 40% or greater in women is likely caused by iron overload

71
Q

The most common cause of hereditary hemochromatosis is caused by what mutation

A

HFE gene

Many clinicians consider tissue biopsy as the “gold standard” for diagnosi

72
Q

The most common abnormalities associated with VTE in pregnancy

A

FVL and PGM

73
Q

Normal physiologic changes in pregnancy

A

(a) increased levels of fibrinogen and factors II, VII, VIII, and X;

(b) decreased protein S activity from reductions in total and free protein S antigen

(c) decreased protein C activity because of decreased activity of protein S (a cofactor for protein C) and increased factor VIII activity

(d) increased levels and activity of the fibrinolytic inhibitors, thrombin-activatable fibrinolytic inhibitor, and plasminogen activator inhibitor types 1 and 2

2-7-8-10, S

74
Q

TRUE OR FALSE

As D-dimer levels rise with a normal pregnancy, they may not be useful in excluding VTE; in such case, diagnostic imaging is required.

A

TRUE

As D-dimer levels rise with a normal pregnancy, they may not be useful in excluding VTE; in such case, diagnostic imaging is required.

75
Q

TRUE OR FALSE

All women with a prior history of VTE should be offered postpartum prophylaxis; those with a high risk of recurrence should receive antepartum prophylaxis.

A

TRUE

All women with a prior history of VTE should be offered postpartum prophylaxis; those with a high risk of recurrence should receive antepartum prophylaxis.

76
Q

A life-threatening condition of pregnancy in which a consumptive thrombocytopenia is accompanied by microangiopathic hemolysis and hepatic dysfunction.

Most patients are diagnosed between 27 and 37 weeks of pregnancy

A

HELLP syndrome

77
Q

Risk factors for HELLP

A
  • European ancestry
  • Multiparity
  • Maternal age older than 34 years
  • Personal or familiar history of HELLP syndrome
78
Q

The most common presenting symptom of HELLP

A

Abdominal pain

WILLIAM'S HEMATOLOGY (109 decks)

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