Pediatrics: Hematologic Conditions Flashcards
A patient presents with:
Pancytopenia progressing to Severe Aplastic Anemia
bone-marrow hypoplasia/aplasia
Congenital Malformation
What is this concerning for?
Fanconi Anemia
What is fanconi anemia often misdiagnosed as?
ITP
A patient presents with:
Pica
Mild anemia sxs
CBC:
Microcytic/Hypochromic Anemia
Mild < Hb
Ferritin < 12
IDA
What is the treatment regimen for IDA?
iron supplementation 2 mg/kg/d PO q 3x daily
A patient has the following finding on peripheral smear:
hypersegmented nuclei
macroovalocytes
Folate/B12 deficiency
A patient presents with the following:
Hemolytic Anemia S/S
(+) Spherocytes
(+) osmotic fragility
Hereditary Spherocytosis
What is the tx for hereditary spherocytosis?
supportive, +/- splenectomy
A patient presents with the following:
Microcytic anemia w/ normal RDW
Anemia Sxs
thalassemia
What is definitive dx for thalassemias?
Hb electrophoresis
What is the treatment for thalassemias
iron monitoring and chelation
splenectomy
What is the treatment for sickle cell?
avoidance of precipitating factors
hydroxyurea
stem cell transplant
A patient presents with:
Jaundice
Hyperbilirubinemia
(+) Bite Cells
(+) Heinz Bodies
G6PD
A patient presents with:
Mild anemia S/S
Normocytic anemia
Basophilic stippling
This is concerning for…
Lead Poisoning
What is the tx for lead poisoning?
chelation
Extremely high Hb
Profound erythrocytosis
Plethora
Splenomegally
This is concerning for…
Familial Polycethemia
This condition presents with:
Profound erythrocytosis
cyanotic heart disease
chronic pulmonary disease
Polycythemia Secondary
How do you treat polycythemia?
phlebotomy
A patient presents with:
Hx of Viral Infx Petechiae Ecchymosis epistaxis Thrombocytopenia
ITP
ITP is a diagnosis of…
exclusion
What should be avoided with ITP?
NSAIDs, ASA, platelet altering medications
What is the prognosis for ITP?
90% spontaneous remission
This condition presents with:
Prlonged mucosal bleeding
Easy bruising
Prolonged bleeding time
vWB disease
How is von willebrand disease treated?
desmopressin
A patient presents with:
Spontaneous Hemarthrosis
Labs:
Prolonged aPTT
Normal vWF, platelets, PT, Bleeding time
Hemophilia
This hemophilia is MC, and is a factor VIII issue
Hemophilia A
This type of hemophilia is called christmas disease and is a factor IX issue…
hemophilia B
How is hemophilia A treated?
Desmopressin
A patient presents with:
Signs of diffuse bleeding:
- purpura
- hematuria
- Melena
- Petechiae
Thrombotic lesions:
-purpura fulminans
This is concerning for…
DIC
The following test results indicate what condition?
elevated D-Dimer and FDP
Prolonged aPTT/PT
Reduced platelets and fibrinogen
DIC
How is DIC treated?
Tx of trigger
Replacement therapy
Anticoagulation if indicated
which type of disorders have the following presentation:
Hypercoagulable state
Increased thrombosis
DVT/PE
FHx of thrombosis
Thrombitic disorders
Protein C Deficiency
Protein S deficiency
Factor V leiden deficiency
Antithrombin deficiency
These are all…
thrombotic disorders
How are thrombotic disorders treated?
(Anticoagulation)
UFH
LMWH
Warfarin
This thrombotic disorder can cause warfarin induced skin necrosis…
protein C deficiency
This protein inactivates factors V and VIII
Protein C
This is a protein C cofactor…
Protein S
Deficiency in this causes increased risk for VTE and makes platelets resistant to inactivation
Factor V leiden
This is an inhibitor of thrombin, and causes decreased heparin efficiency
Antithrombin
A patient presents with:
Preceding viral URI
Palpable, non-blanching purpura
Arthritis/Arthralgia
Abdominal pain
Renal Disease
Deposition of IgA complexes…
HSP
A patient has the following lab results:
CBC:
Normal/elevated platelets
Elevated ASO Titer, IgA
(+) Hemoccult
UA:
- Hematuria
- Proteinuria
What is this concerning for
HSP
What is the Tx for HSP?
Supportive
