Pediatrics: Hematologic Conditions Flashcards

1
Q

A patient presents with:

Pancytopenia progressing to Severe Aplastic Anemia

bone-marrow hypoplasia/aplasia

Congenital Malformation

What is this concerning for?

A

Fanconi Anemia

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2
Q

What is fanconi anemia often misdiagnosed as?

A

ITP

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3
Q

A patient presents with:

Pica
Mild anemia sxs

CBC:
Microcytic/Hypochromic Anemia
Mild < Hb
Ferritin < 12

A

IDA

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4
Q

What is the treatment regimen for IDA?

A

iron supplementation 2 mg/kg/d PO q 3x daily

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5
Q

A patient has the following finding on peripheral smear:

hypersegmented nuclei
macroovalocytes

A

Folate/B12 deficiency

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6
Q

A patient presents with the following:

Hemolytic Anemia S/S
(+) Spherocytes
(+) osmotic fragility

A

Hereditary Spherocytosis

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7
Q

What is the tx for hereditary spherocytosis?

A

supportive, +/- splenectomy

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8
Q

A patient presents with the following:

Microcytic anemia w/ normal RDW
Anemia Sxs

A

thalassemia

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9
Q

What is definitive dx for thalassemias?

A

Hb electrophoresis

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10
Q

What is the treatment for thalassemias

A

iron monitoring and chelation

splenectomy

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11
Q

What is the treatment for sickle cell?

A

avoidance of precipitating factors

hydroxyurea

stem cell transplant

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12
Q

A patient presents with:

Jaundice
Hyperbilirubinemia
(+) Bite Cells
(+) Heinz Bodies

A

G6PD

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13
Q

A patient presents with:

Mild anemia S/S
Normocytic anemia
Basophilic stippling

This is concerning for…

A

Lead Poisoning

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14
Q

What is the tx for lead poisoning?

A

chelation

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15
Q

Extremely high Hb
Profound erythrocytosis
Plethora
Splenomegally

This is concerning for…

A

Familial Polycethemia

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16
Q

This condition presents with:

Profound erythrocytosis
cyanotic heart disease
chronic pulmonary disease

A

Polycythemia Secondary

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17
Q

How do you treat polycythemia?

A

phlebotomy

18
Q

A patient presents with:

Hx of Viral Infx
Petechiae
Ecchymosis
epistaxis
Thrombocytopenia
A

ITP

19
Q

ITP is a diagnosis of…

A

exclusion

20
Q

What should be avoided with ITP?

A

NSAIDs, ASA, platelet altering medications

21
Q

What is the prognosis for ITP?

A

90% spontaneous remission

22
Q

This condition presents with:

Prlonged mucosal bleeding
Easy bruising
Prolonged bleeding time

A

vWB disease

23
Q

How is von willebrand disease treated?

A

desmopressin

24
Q

A patient presents with:
Spontaneous Hemarthrosis

Labs:
Prolonged aPTT

Normal vWF, platelets, PT, Bleeding time

A

Hemophilia

25
Q

This hemophilia is MC, and is a factor VIII issue

A

Hemophilia A

26
Q

This type of hemophilia is called christmas disease and is a factor IX issue…

A

hemophilia B

27
Q

How is hemophilia A treated?

A

Desmopressin

28
Q

A patient presents with:

Signs of diffuse bleeding:

  • purpura
  • hematuria
  • Melena
  • Petechiae

Thrombotic lesions:
-purpura fulminans

This is concerning for…

A

DIC

29
Q

The following test results indicate what condition?

elevated D-Dimer and FDP

Prolonged aPTT/PT

Reduced platelets and fibrinogen

A

DIC

30
Q

How is DIC treated?

A

Tx of trigger

Replacement therapy

Anticoagulation if indicated

31
Q

which type of disorders have the following presentation:

Hypercoagulable state

Increased thrombosis

DVT/PE

FHx of thrombosis

A

Thrombitic disorders

32
Q

Protein C Deficiency
Protein S deficiency
Factor V leiden deficiency
Antithrombin deficiency

These are all…

A

thrombotic disorders

33
Q

How are thrombotic disorders treated?

A

(Anticoagulation)
UFH
LMWH
Warfarin

34
Q

This thrombotic disorder can cause warfarin induced skin necrosis…

A

protein C deficiency

35
Q

This protein inactivates factors V and VIII

A

Protein C

36
Q

This is a protein C cofactor…

A

Protein S

37
Q

Deficiency in this causes increased risk for VTE and makes platelets resistant to inactivation

A

Factor V leiden

38
Q

This is an inhibitor of thrombin, and causes decreased heparin efficiency

A

Antithrombin

39
Q

A patient presents with:

Preceding viral URI

Palpable, non-blanching purpura

Arthritis/Arthralgia

Abdominal pain

Renal Disease

Deposition of IgA complexes…

A

HSP

40
Q

A patient has the following lab results:

CBC:
Normal/elevated platelets

Elevated ASO Titer, IgA

(+) Hemoccult

UA:

  • Hematuria
  • Proteinuria

What is this concerning for

A

HSP

41
Q

What is the Tx for HSP?

A

Supportive