Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

COM Heme and Lymph Exam 1 > Exam 2 > Flashcards

Exam 2 Flashcards

1
Q

Prothrombin Time (PT)

A

Assesses the extrinsic system. Normal: 10-13 sec.

Prolonged in deficiencies of Factors II, V, IVV, X, fibrinogen, patients taking warfarin or dicoumarol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Partial Thromboplastin Time (PTT)

A

Assesses the intrinsic system. Normal 25-40 sec.

Prolonged in deficiencies of Factors VIII, IX, XI, XII, patients on heparin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Thrombin Time (TT)

A

Assesses for deficiency or abnormalities in fibrinogen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Platelet Aggregation Studies

A

Assist in diagnosis of vWF disease, storage pool disease, Bernard-Soulier syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the most likely cause of petechiae and thrombocytopenia?

A

Medications

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

DIC

A

Med/surg/obstetrical complication. Initial thrombosis as intrinsic/extrinsic coagulation systems activated. Depletion of platelets and clotting factors leads to bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Treatment of DIC

A

Treat the underlying disorder! Supportive care

Heparin only with overt thrombosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

TTP

A

Generalized disorder of microcirculation; TTP, microangiopathic hemolytic anemia, fluctuating neurological signs, renal dysfunction, and febrility

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

HUS

A

MAHA + Thrombocytopenia (<50K) + fever + neurologic symptoms + RENAL FAILURE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Signs/Symptoms of TTP

A

Microangiopathic anemia - shistocytes, helmet cells

Pathologic lesion - hyaline thrombi occluding capillaries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

2 major forms of TTP

A
  1. Hereditary - ADAMTS13 gene mutation

2. Acquired - autoAbs directed at ADAMTS13

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Treatment of TTP

A

Treat underlying disorder.

Plasmapharesis is life saving in almost all cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

von Willebrand disease

A

Spectrum of diseases with decreased platelet adhesion to vascular endothelium (mediated by vWF).
Decreased/absent production of vWF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Results of platelet aggregation tests in von Willebrand Disease

A

Abnormal - espeically to ristocetin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Treatment for von Willebrand disease

A

DDAVP (desmopressin) - type I, release of vWF from endothelium
Cryoprecipitate - type 2 and 3, replaces vWF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Glanzmann’s thrombasthenia

A

Inherited (AR) qualitative platelet disorder

Absence of platelet Gp IIb/IIIa receptor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Bernard-Soulier syndrome

A

Inherited (AR) qualitative platelet disorder

Absence of platelet Gp Ib-IX-V receptor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Result of platelet aggregation studies for Glanzmann’s thrombasthenia

A

Restocetin is present, but all other aggregation studies are absent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Result of platelet aggregation studies for Bernard-Soulier syndrome

A

Restocetin is ABSENT, all other aggregation studies normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Platelet Storage Pool Disorder (SPD)

A

Autosomal dominant qualitative platelet disorder

Mild bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Hermansky-Pudlak syndrome

A

Inherited disorder of granule formation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Treatment of severe platelet dysfunction

A

Platelet transfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Reasons for acquired disorders of platelet function

A

Medications, uremia, cardiopulm bypass, myelodysplastic or myeloproliferative syndromes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Hemophilia A

A

X-linked recessive trait, deficiency of Factor VIII
Risk of bleeding corresponds to degree of deficiency
Mild, Moderate, or Severe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Clinical features of Hemophilia A

A

Easy bleeding/bruising, hematomas, frequent hemarthroses

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Hemophilia B

A

Decreased serum Factor IX

Similar inheritance, course, clinical features as A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Deficiency of vitamin K-dependent factors

A

Features: bleeding/hemorrhage, prolonged PT, deficiency of factors II, VII, IX, X, Proteins C and S

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Hereditary Hemorrhagic Telangiectasia AKA

Osler-Weber-Rend syndrome

A

AD inheritance, gene defect in endoglin (c9)
Caused by thinning of vessel walls with telangiectatic formations, AV malformations, and aneurysmal dilations throughout the body

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Clinical features of Hereditary Hemorrhagic Telangiectasia

A

Benign clinical course, recurrent bleeds frequent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Treatment of Hereditary Hemorrhagic Telangiectasia

A

Surgery and laser photoablation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Anti-Thrombin III Deficiency (AT-III)

A

Clinical: recurrent LE thrombophlebitis and DVT, venous insufficiency, chronic leg ulcers
Significantly increased DVT risk in pregnancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

How do you diagnose AT-III Deficiency

A

Diminished levels of AT-III in serum (<50% activity)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Treatment of AT-III Deficiency

A

Prophylactic tx with anticoagulants, patients with DVT should receive heparin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Deficiency of Protein C and S

A

Protein C-inactivates factors V and VIII

Protein S-cofactor for protein C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Treatment of Protein C and S

A

Warfarin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Factor V Leiden mutation

A

Abnormality of factor V at binding site for activated protein C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Treatment of Factor V Leiden mutation and Prothrombin 20210

A

No prior episodes: monitor; DVT prophylaxis

Prior episodes: consider lifelong anticoagulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Prothrombin 20210

A

G-A mutation resulting in increased activity for prothrombin and inability to deactivate prothrombin
HIGH risk throbosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Antiphospholipid Syndrome

A

Circulating antibodies to phospholipid

Associated with: anticardiolipin antibody syndrome, lupus anticoagulant, false positive VDRL Ab syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Features of Antiphospholipid syndrome

A

Thromboembolic phenomena, miscarriage, thrombocytopenia, cerebral ischemia and recurrent stroke, UBO
CT disease, prolonged PTT that fails to correct with mixing studies, valvular heart disease, CAD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Diagnosis of Antiphospholipid syndrome

A
  1. Prolonged PTT
  2. Lack of correction in mixing studies
  3. Neutralization of inhibitor with excess phospholipid
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Test specific for lupus anticoagulant

A

DRVVT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Treatment of Antiphospholipid syndrome

A

No benefit of tx if no hx thromboembolic dz
Hx of dz: lifelong anticoagulation
Hydroxychloroquine-thromboembolism reduction in patients with APS, SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

How to exclude pseudothrombocytopenia

A

Repeat CBC with heparin or citrate tube

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Erythromelalgia

A

Vasomotor changes that occur in patients with essential thrombocythemia, may be accompanied by burning pain
Treatment: daily baby ASA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Common drug causes of lowered platelet count?

A

Loop diuretics, H2 blockers, digoxin, Abx

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Acute ITP

A

ISOLATED thrombocytopenia in absence of underlying causes - children otherwise appear well
Peak incidence 2-5 yrs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Difference between Acute and Chronic ITP

A 
Acute = up to 6 months
Chronic = greater than 6 months
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Treatment of Acute ITP?

A

Generally - no tx alters natural history of dz

Some treat - prednisone, IVIG, anti-D Ig

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Red Flags in Peds and what do they mean?

A

Red flags: constitutional sx, hx disease with low platelets, dietary hx suggestive of deficiency, exposure to meds assoc with low platelets, PE findings other than bleeding
These red flags r/o ITP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Chronic ITP

A

Sx last longer than 6 mos; generally benign and mostly otherwise well
Does not require aggresive drug tx
Platelet counts 30-80K
Can attend school and participate EXCEPT CONTACT SPORTS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Treatment of chronic ITP

A

Only cure is splenectomy, but this is approached cautiously

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Myelodysplastic syndrome

A

Disorder of pluripotential stem cell leading to ineffective hematopoiesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Clinical features of myelodysplastic syndromes

A

Pancytopenia with hyperplastic marrow, potential risk for development of acute leukemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Etiology of myelodysplastic syndromes

A

Radiation, petrochemical exposure, chemotherapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Cytogenetic markers of myelodysplastic syndromes

A

Partial loss of long arm chromosome 5 or 7
Inv(16)
Trisomy 8

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Laboratory abnormalities in myelodysplasia

A

Elevated LDH, serum ferritin

Normal serum Fe, TIBC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Most common causes of pancytopenia

A

Hypersplenism, aplastic anemia, myelodysplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Prognosis and adverse prognostic features in myelodysplasias

A

Poor prognosis
Features: more than 5% marrow blasts, platelet count lower than 100k, Hb lower than 10g/dl, neutrophils lower than 25k, age greater than 60 yrs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

Favorable prognostic feature in myelodysplasias

A

5q syndrome - beneficial response to lenalidomide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Treatment of myelodysplasia

A

Supportive - avoid medications that damage marrow, aggressive treatment of infection, transfuse PRBCs if symptomatic, transfuse platelets for bleeding or surgery propylaxis, monitor for iron overload
EPO, androgens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Low/Intermediate intensity therapy

A

Hypomethylating agets, lenalidomide for 5q syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

High intensity therapy

A

AML induction treatment, stem cell transplantation

64
Q

Myelofibrosis

A

Primary myeloproliferative d/o characterized by marrow fibrosis and extramedullary hematopoiesis

65
Q

Triad of symptoms in myelofibrosis

A

Leukoerythroblastic anemia, poikilocytosis, splenomegaly

66
Q

Disease course of myelofibrosis

A

Chronic but progressive, patient may be asymptomatic for years; progressive pancytopenia and organomegaly indicate development to later stage of disease

67
Q

Treatment for myelofibrosis

A

Observation if patient asymptomatic
Otherwise, treat with BMT
Splenomgaly?
Allogenic stem cell transplant the only cure

68
Q

Neutropenic fever

A

Temp above 38.5 C (101.4 F) with ANC <500

69
Q

Most common pathogens associated with neutropenic fever

A

Bacteria: S. aureus, S. epidermis, Klebsiella
Fungi: CANDIDA, Aspergillus, Pneumocystis, Toxo

70
Q

What should you NOT do in a pt with severe neutropenia/neutropenic fever?

A

Rectal exam/genitourinary exams

71
Q

Primary Prophylaxis for pts at risk for neutropenic fever

A

Antibacterial: Fluoroquinolones (floxacins) to target P. aeruginosa and gram - bacilli
Antifungal: Fluconazole (candida) or echinocandins (-fungins) for more broad coverage

72
Q

Secondary Prophylaxis for pts with history of fungal infection or high infection risk

A

Voriconazole: 1st line for Aspergillus

73
Q

Adverse effects of Fluoroquinolones

A

Prolonged QT interval, tendon rupture, increased risk C. diff infection

74
Q

Contraindications with Voriconazole?

A

DON’T GIVE with: cytarabine, fludarabine, vincristine

75
Q

Inpatient Empiric IV Antibiotics

A

Piperacillin + tazobactam
Carbapenem
Ceftazidime
Cefepime

76
Q

Empiric IV antibiotic for cellulitis or pneumonia

A

Vancomycin or linezolid

77
Q

Empiric IV antibiotic for gram negative bacteremia

A

Add aminoglycoside (ex: gentamicin)

78
Q

Empiric IV antibiotic for C. difficile

A

Metronidazole

79
Q

Empiric antifungal and when do you add it?

A

Caspofungin; add if fever does not resolve after 5 days of abx tx

80
Q

Non-antibiotic/anti-fungal considerations for neutropenic fever

A

Adequate hydration

G-CSF (Filgrastim)

81
Q

Most common cancer in adolescents?

A

Lymphoma

82
Q

Most common malignancy of age group 15-19?

A

Hodgkin’s lymphoma

83
Q

What cells are pathognomic for Hodgkin’s lymphoma?

A

Reed-Sternberg cells

84
Q

What are the B symptoms?

Why are they significant?

A

Sx: unexplained fever, weight loss more than 10% body weight over 6 months, drenching night sweats
Importance: Staging HL

85
Q

1st diagnostic test for patient with persistent unexplained LAD without obvious underlying infection process?

A

CXR! THEN - LN biopsy

86
Q

Which neoplasm responsible for 60% lymphomas in children/adolescents?

A

Non-Hodgkin’s lymphoma

87
Q

Name the syndrome - X-linked recessive d/o with recurrent sinopulmonary and ear infections, severe atopic dermatitis, and bleeding d/t thrombocytopenia

A

Wiscott-Aldrich syndrome

88
Q

Burkitt Lymphoma

A

Sporadic type - abdominal mass, Endemic - head/neck

+ Involvment of bone marrow or CNS

89
Q

Factors that affect prognosis of Non-Hodgkin’s lymphoma

A

Localized disease: 90-100% survival
Advanced disease: 60-95% survival
Depends on pathologic subtype, tumor burden at dx (serum LDH), CNS disease, metastasis

90
Q

Neoplasm that constitutes 97% of all childhood leukemias

A

Acute leukemia

91
Q

Chromosomal abnormality that is highly associated with ALL and AML (at less than 3 y/o)

A

Trisomy 21

92
Q

Which way does the O2 dissociation curve shift in anemia?

A

RIGHT

93
Q

MCV

A

Mean Corpuscular Volume. Mean value of individual RBC volume in sample; measured by automated blood counters

94
Q

MCHC

A

Mean Corpuscular Hgb Concentration. Index = Hgb/Hct

Grams of Hgb per 100 mL RBCs

95
Q

MCH

A

Average content (mass, weight) of Hgb per RBC

96
Q

RDW

A

Red cell Distribution Width; quantitative measure of variability of RBC sizes in sample (anisocytosis)

97
Q

Specific features of intravascular hemolysis

A

Hemoglobinuria, hemoglobiemia, hemosiderinuria

98
Q

Specific features of extravascular hemolysis

A

SPLENOMEGALY, anemia, jaundice

99
Q

Specific feature in sequestration

A

BLEEDING

100
Q

What is the meaning of a low-normal reticulocyte count?

A

Inadequate bone marrow response to anemia

101
Q

Main causes of MICROCYTIC anemia?

A

Iron deficiency, Lead intoxication, thalassemias

102
Q

Physiologic nadir of hemoglobin in infants?

A

At 2-3 months of age. Hgb=9-11, Hct=28-35%

103
Q

Isoimmunization

A

Neonatal cause of anemia d/t ABO or Rh incompatibility

104
Q

Test results associated with isoimmunization

A

+ Direct Coomb’s, increased indirect bilirubin, normocytic anemia, increased reticulocyte count

105
Q

Heinz bodies and Bite cells are seen with what deficiency?

A

G6PD Deficiency

106
Q

Most likely viral cause of neonatal anemia?

A

PARVOVIRUS

107
Q

Diamond-Blackfan syndrome

A

CONGENITAL PURE RED CELL APLASIA-congenital macrocytic anemia with decreased reticulocytes; d/t apoptosis of erythroid precursors

108
Q

Fanconi Anemia

A

Macrocytic anemia, leads to PANCYTOPENIA

109
Q

Primary cause of iron deficiency anemia in toddlers?

A

Inadequate dietary intake - heavy milk drinkers

110
Q

Disorder of Hgb structure/synthesis

A

Causes microcytic anemia, decreased RDW; LOW MENTZER INDEX IN THALASSEMIA

111
Q

Lead poisoning risk factors

A

Young age, houses built before 1970s, contaminated soil, pica

112
Q

Features of anemia associated with lead poisoning

A

Microcytic anemia, peripheral smear may show basophilic stippling

113
Q

Calculate Absolute Neutrophil Count (ANC)

A

((% neutrophils + % bands) x (WBC)/100)

114
Q

ANC in Neutropenia? Define mild, moderate, and severe neutropenia in terms of ANC

A

Neutropenia: ANC < 1500
Mild: ANC is 1000-1500
Moderate: ANC is 500-1000
Severe: ANC is less than 500

115
Q

Severe Congenital Neutropenia (Kostmann Syndrome)

A

Autosomal recessive, d/t impaired myeloid differentiation caused by mutational arrest of neutrophil precursors
Life-threatening pyogenic infx in infancy
Increased risk of AML

116
Q

Cyclic Neutropenia

A

Autosomal dominant, defect in stem cell regulation leads to defective maturation
Cyclic fever, oral ulcers, gingivitis, periodontal disease, recurrent bacterial infx, NO increased risk of malignancy

117
Q

Schwann-Diamond Syndrome

A

Autosomal recessive, Neutropenia + defects in neutrophil mobility, migration, chemotaxis
Triad: 1. Neutropenia, 2. Exocrine pancreas insufficiency, 3. Skeletal abnormalities
Increased risk for myelodysplastic syndrome/leukemia

118
Q

Fanconi Anemia

A

“Classic” congenital neutropenic disorder
Bone marrow failure, GU+skeletal abnormalities, increased chromosome fragility, autosomal recessive
Presents 1-10 yrs of age

119
Q

Leukocyte Adhesion Deficiency

A

Very rare, autosomal recessive disorder
Delayed separation of umbilical cord (>3 wks), recurrent and severe bacterial/fungal infx w/o pus
Neutrophils have diminished adhesion to surfaces

120
Q

Chronic Granulomatous Disease

A

X-linked recessive, 1:250000
Recurrent purulent infx w/ fungal or bacterial catalase + organisms, usually starts in infancy, granulomas
Defective oxidative metabolism, no generation of superoxide - good prognosis

121
Q

Infection Associated Neutropenia

A

Viral cause w/in 2-3 days of illness to 1 week after

MOST common: Parvovirus B19 - Fifth’s disease with “slapped cheek appearance” after 1-2 weeks

122
Q

Acquired Neutrophil Disorders

A

Due to cytotoxic agents (chemo), Vit B12 deficiency

123
Q

Causes of leukocytosis

A

Reactive due to infection
Chronic inflammation (autoimmune disorders)
Oncologic process
Seizure

124
Q

Most common trisomy associated with defective maturation or proliferation of myeloid cells

A

TRISOMY 21 - DOWN SYNDROME

125
Q

Acute vs. Chronic Leukemia in terms of cell types

A

Acute: proliferation of immature blast cells
Chronic: proliferation of mature appearing cells

126
Q

Toxins and drugs associated with development of leukemia (specify type)

A
  1. Benzene - AML
  2. Chemotherapy (alkylators) - treatment related AML
  3. Radiation - all leukemias EXCEPT CLL
  4. Viruses - HTLV-1 in T cell lymphoma
  5. Hereditary disease
  6. Myelodysplastic syndromes
127
Q

Most common diseases associated with pancytopenia and macrocytosis?

A

Myelodysplastic syndrome or early AML

128
Q

Most common chromosomal aberration (pathognomonic) in CML

A

Philadelphia chromosome t(9;22) = BCR-ABL fusion gene

129
Q

Compare clinical presentation of acute vs. chronic leukemia

A

Acute: rapid onset and course
Chronic: slow onset, chronic course

130
Q

Most common symptom/complaint of patients with acute leukemias (esp ALL)

A

Bone pain

131
Q

Sweet’s Syndrome

A

Acute Febrile Neutrophilic Dermatosis (AFND)

Cutaneous manifestation of AML (myeloblasts in dermis)

132
Q

Auer rods

A

Purple rods seen in cytoplasm of patients with AML (most commonly APL)

133
Q

Oral manifestation in M4 and M5 variants of AML?

A

Gingival hypertrophy

134
Q

In what setting are smudge cells seen?

A

CLL (multiple are seen)

135
Q

Describe findings of affected lymph nodes in a lymphoma

A

Rubbery, non-tender

136
Q

Associated with pain in lymph nodes after beer drinking?

A

Hodgkin’s lymphoma

137
Q

Richter’s syndrome

A

Patients with pre-existing CLL develop DLBCL

138
Q

B symptoms

A

Fever, drenching night sweats, 10% weight loss in previous 6 months - Paraneoplastic, worse px

139
Q

Top DDx for patients with cervical LAD

A

Infection, lymphoma, unusual disorders (Kikuchi)

140
Q

What kind of patients at high risk for primary CNS lymphoma?

A

IMMUNOSUPPRESSED (HIV, organ transplant, etc)

141
Q

Major cause of neutrophilia and neutropenia?

A

Drugs

142
Q

Major causes of neutropenia in patients?

A

Medications, Nutritional deficiences, Sequestration

143
Q

Waldenstrom’s Macroglobulinemia

A

Malignancy of lymphoplasmacytoid cells secreting IgM.
Major clinical manifestation: HYPERVISCOSITY
MYD88 mutation, slightly more common in older men
May develop peripheral neuropathy, visual disturbance
Kappa light chain excretion.
Rouleau formation and + Coombs test

144
Q

Treatment of Waldenstroms Macroglobulinemia

A

Plasmapheresis

Bortezomib, bendamustine, Rituximab

145
Q

POEMS syndrome

A

Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes
Hepatomegaly, LAD
Circulating pro-inflammatory cytokines

146
Q

Treatment of POEMS syndrome

A

Radiotherapy

147
Q

Gamma Heavy Chain Disease (Franklin’s Dz)

A

LAD, fever, anemia, HSM, weakness, palatal edema
Association with autoimmune diseases
Diagnosis based on anomalous serum M component
Thrombocytopenia, eosinophilia, non-dx bone marrow
Therapy when symptomatic = chemo, rituximab

148
Q

Alpha Heavy Chain Disease (Seligmann’s Dz)

A

Most common of heavy chain diseases
CHRONIC DIARRHEA, wieght loss, malabsorption
LAD of mesenteric and paraaortic nodes
Antibiotics or chemotherapy if unresponsive

149
Q

Mu Heavy Chain Disease

A

Very rare, occurs with CLL
Vacuoles in malignant lymphocytes and excretion of kappa light chains in the urine
Treat as you would treat CLL

150
Q

Plasma cell dyscrasias

A

Clonal proliferation of IG-secreting plasma cells

151
Q

Hallmark feature of plasma cell dyscrasias

A

Isolated peak in the gamma region on serum protein electrophoresis representing a SINGLE complete immunoglobulin molecule (M-protein)

152
Q

Likely diagnosis with A:G ratio <1

A

Plasma cell disorder (MGUS, myeloma, etc)

153
Q

Multiple Myeloma features

A

Triad: marrow plasmacytosis >10%, lytic bone lesions, serum or urine M component
Hypercalcemia, hypogammaglobulinemia

154
Q

Treatment of multiple myeloma

A

Thalidomide (-imib) + Dexamethasone

Biphosphonates to control hypercalcemia

155
Q

Treatment of Waldenstrom’s Macroglobulinemia

A

Pentastatin, 2CDA

COM Heme and Lymph Exam 1 (2 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions