Hematology Flashcards

1
Q

Hallmarks of primary erythrocytosis (polycythemia Vera)

A

Facial flushing, pruritis (esp. after hot shower), splenomegaly, engorged retinal veins, headache.

*treat with phlebotomy

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2
Q

Organs effected in Hemachromatosis

A

Liver: bronze skin
Pancreas: DM
Heart: Restrictive cardiomyopathy
Endocrine: hypergonadism

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3
Q

S/S of iron deficiency anemia

A
  • pagophagia (ice craving)
  • pica
  • angular cheilitis
  • koilonychia (spooning of nails)
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4
Q

Diagnostic lab findings in iron deficiency anemia

A
  • Low iron
  • high TIBC
  • LOW ferritin
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5
Q

Diagnostic lab findings in anemia of chronic disease

A
  • low total iron
  • HIGH ferritin (think all the iron is sequestered in storage)
  • low TIBC
  • high hepcidin
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6
Q

Rouleax formation on smear is associated with

A

Multiple myeloma

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7
Q

Auto agglutination on smear is associated with

A

Cold agglutination autoimmune hemolytic anemia

MYCOPLASMA PNEUMONIA , EBV

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8
Q

Howell-jolly bodies associated with

A

-decreases splenic function AKA sickle cell dz

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9
Q

Bite cells on smear associated with

A

Thalassemia, G6PD

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10
Q

Basophillic stippling is associated with

A

Sideroblastic anemia-lead poisoning

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11
Q

Auer rods are associated with

A

Acute myelogenous leukemia

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12
Q

Reed stern berg cells associated with

A

Hodgkin lymphoma

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13
Q

Pathophysiology of G6PD deficiency

A

Episodic, Nomocytic anemia

*G6PD protects RBC against oxidative stress, when there is a deficiency the hgb is denatured and is precipitated as HEINZ bodies

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14
Q

G6PD deficiency causes

A

1) infection
2) fava beans
3) meds: sulfa drugs, Bactrim, antimalarial

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15
Q

Dx of G6PD deficiency

A

Acute, episodic, hemolytic anemia

  • bite cells
  • heinz bodies
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16
Q

G6PD management

A
  • self limited
  • folate/iron supplementation
  • blood transfusions
17
Q

Management of sickle cell dz

A

1) IV fluids and oxygen for pain control
2) hydroxyurea
3) folic acid

18
Q

Pentad of thrombotic thrombocytopenia purpura

A

1) thrombocytopenia (petechiae, mucocutaneous bleed)
2) neuro s/s
3) microangiopathic hemolytic anemia
4) kidney failure
5) fever

19
Q

Pathophysiology of TTP

A

Antibodies against ADAMTS13 causes a low level. ADAMTS13 is used to cleave vWF, so with this low level there is a lot of free vWF resulting in platelet sequestration

20
Q

TTP treatment

A

Plasmaphoresis

21
Q

Hemophilia A is a deficiency in what factor?

A

8

22
Q

Hemophilia B is a deficiency in what factor

A

9

*christmas dz

23
Q

Hemophilia A treatment

A
  • Factor VIII infusion

- Desmopressin (DDAVP infusion)

24
Q

Howell Jolly Bodies are found in what disease and what do they signify?

A

Sickle cell anemia, they signify a damaged or absent spleen

25
Q

What is ITP?

A

Idiopathic (autoimmune) throbocytopenic purpura:

-Abnormally low platelet count with normal coagulation studies for no know reason ; often ASX

  • Often precipitated by a viral illness
  • Autoantibody reaction against platelets
  • IVIG, steroids, splenectomy is the order of treatmetnt
26
Q

Target cells (codocytes) are found in what diseases?

A

Hemoglobinopathies such as sickle cell anemia or thalassemia

27
Q

What blood disorders are hypo segmented neutrophils found in?

A

Macrocytic anemia, Vit. B12 & folate deficiency

28
Q

Potential causes of Vit. B12 deficiency

A
  1. Pernicious anemia: lack of intrinsic factor from gastric parietal cells
  2. Strict Vegan diet
  3. Malabsorption 2/2 alcoholism
29
Q

Lab findings in vitamin b 12 deficiency

A
  • Hypersegmented neutrophils
  • INC serum homocysteine, methylmalonic acid levels
  • LOW b12
30
Q

Clinical manifestations of sickle cell disease

A

1) dactylitis mC 1st presentation at 6-12 months old
2) Infections: osteomyelitis 2/2 salmonila or other encapsulated organisms, Aplastic crisis associated with parvovirus B19 infections
3) “H” shaped vertebra
4) Splenic sequestration crisis: splenomegaly, rapidly decreasing hemoglobin
5) bone pain and priapism from occlusion of schyistocytes

31
Q

Hallmarks of Hodgkin’s lymphoma

A
  • neoplasm of lymphocytes often presenting before 20 y/o or after 50 y/o
  • Associated with EBV
  • Painless lymphadenopathy and Systemic “B” s/s
  • Involves more central lymph nodes such as neck, axiallary, mediastinal
  • Reed sternberg cells on excision bx is pathopneumonic (owl’s eye appearance)
32
Q

Hallmarks of Non Hodgkin Lymphoma

A
  • Risk factors are increased age and immunosuppression
  • Painless lymphadenopathy of peripheral lymph nodes with extra nodal sites common (GI, skin, CNS)
  • Unpredictable course as it effects B cells, T cells, and follicular cells
  • If indolent follicular form you can use Rituximab (antibody vs. CD20 on B cells) to treat
  • Poor prognosis
33
Q

Acute Lymphocytic Leukemia Hallmarks

A
  • MC childhood malignancy
  • Presents with Fever (MC) bone pain, CNS symptomslethargy, hepatosplenomegaly, lymphadenopathy
  • Bone marrow is hypo cellular with greater than 20% blasts
  • Highly response to combination chemotherapy and intrathecal methotrexate for CNS disease
34
Q

Chronic lymphocytic leukemia hallmarks

A
  • MC leukemia in adults
  • B cell clonal malignancy
  • Pts are often asymptomatic with fatigue, hepatosplenomegaly
  • Peripheral blood smear shows SMUDGE CELLS
35
Q

Acute myelogenous leukemia hallmarks

A
  • Pancytopenia (anemia, thrombocytopenia, neutropenia ) with a leukostasis (WBC over 100,000)
  • Gingival hyperplasia, bone pain
  • Bone marrow shows AUER RODS
36
Q

Chronic myelogenous leukemia hallmarks

A
  • mostly asx until they develop blastic crisis with splenomegaly
    • PHILIDALPHIA CHROMOSOME (Mix of 9 & 22)
  • WBC over 100,000
37
Q

What is polycythemia vera?

A

Acquired myeloproliferative disorder with overproduction of all 3 myeloid cell lines

-increased RBC, WBC, Plts

38
Q

Clinical manifestations of polycythemia vera

A

thrombosis, pruritis, facial plethora, worse after hot bath, splenomegaly

-decreased EPO levels

39
Q

Treatment of polycythemia vera

A

Phlebotomy