VISUAL DIAGNOSIS VISUAL DIAGNOSIS

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VISUAL DIAGNOSIS VISUAL DIAGNOSIS

VISUAL DIAGNOSIS


A “ spidery ” rash

Lindsay Jackson, MD

October 14, 2009


Case of a “spidery “ spidery” ” rash

3 year old male develops a spidery rash over

the past week on his arms and legs

3 year old male develops a spidery rash over

Bruising noted over lower extremities

Dried blood noted at ears, nose

“Blood “ Blood blister” blister ” on lower lip


Purpura


The “spidery “ spidery” ” rash


Exam

Growth parameters and vital signs normal

Well appearing

HEENT: ecchymosis over left eye, crusted

blood in nares, palatal petechiae

HEENT: ecchymosis over left eye, crusted

Abdomen: no hepatosplenomegaly

Skin: petechiae on arms, back, legs. Pretibial

bruising

Skin: petechiae on arms, back, legs. Pretibial


Differential Diagnosis

What causes thrombocytopenia (platelet count

below 150,000) in children?

What causes thrombocytopenia (platelet count

Idiopathic thrombocytopenic purpura

Drug induced thrombocytopenia (heparin)

TTP

Sepsis/DIC

Congenital

TAR (Thrombocytopenia, absent radii)

Kasabach­ Kasabach ­Merritt Merritt

Wiskott­ Wiskott ­Aldrich Aldrich


Labs

WBC 7.3 (29% neutrophils, 63% lymphocytes,

6% monocytes, 2% eosinophils)

WBC 7.3 (29% neutrophils, 63% lymphocytes,

Hemoglobin 11.9 with Hematocrit 34.4

Platelet count 2000

Chemistry panel

Chemistry panel

Na 132, K 5.6 (Hemolyzed) and LDH 473

Liver function studies normal


Figure 2. Peripheral smear in a patient with ITP showing an almost total absence of platelets

Lazarchick, J. ASH Image Bank 2001;2001:100177

Copyright ©2001 American Society of Hematology. Copyright restrictions may apply.


Idiopathic Thrombocytopenic

Objectives

Purpura (ITP)

Objectives

Know function and life span of platelets

Describe the usual presentation and laboratory

tests in the child with ITP

Describe the usual presentation and laboratory

Review the treatment options

Know the usual prognosis


Normal peripheral smear

Normal platelets are one fifth the

diameter of erythrocytes.

They survive in the body for 9 to 10

days


Idiopathic Thrombocytopenic

Purpura (ITP)

Estimated to be one of the most common

acquired bleeding disorders encountered by

pediatricians

Estimated to be one of the most common

3 to 8 cases per 100,000 children per year

3 to 8 cases per 100,000 children per year

A general pediatrician can care for most

children with ITP

A general pediatrician can care for most


ITP

Autoantibodies (usually IgG) are directed against

platelet membrane antigens

Autoantibodies (usually IgG) are directed against

Antibody­ Antibody ­coated coated platelets have a shortened half­ half ­life life

because of accelerated clearance by tissue

macrophages in the spleen and other portions of the

reticuloendothelial system.

The net effect is a decrease in the platelet count.


IgG coated Platelets


Presentation

Most common in ages 2 to 5

Characterized by the sudden appearance of

bruising/bleeding/petechiae in an otherwise

healthy child

Characterized by the sudden appearance of

Association with prior infection

Association with MMR vaccination


Petechiae in ITP


Pertinent Negatives

No systemic symptoms

Absence of lymphadenopathy and

hepatosplenomegaly on exam

No evidence of hemolysis

Absence of lymphadenopathy and

No evidence of hemolysis


Laboratory findings

Platelet count typically below 30,000

15% of patients will have mild anemia due to

bleeding

Essential to review peripheral smear

15% of patients will have mild anemia due to

Essential to review peripheral smear

Large platelets


Leukemia

Leukemia

What do parents fear?

In one study of 2239 patients with newly

diagnosed ALL, NONE exhibited isolated

thrombocytopenia

Bone marrow examination not necessary in routine

cases

In one study of 2239 patients with newly

Bone marrow examination not necessary in routine

Dubansky AS, Boyett JM, Falletta J, et al: Isolated thrombocytopenia thrombocytop enia in children with

acute lymphoblastic leukemia: A rare event in a Pediatric Oncology Oncolo gy Group study.

Pediatrics 1986;77:49


Chronic ITP

Thrombocytopenia lasting longer than 6

months

Thrombocytopenia lasting longer than 6

Thyroid studies

Thyroid studies

ANA

Coombs test

HIV/CMV/EBV

Immunodeficiency


Pharmacologic Therapy

70 to 80 percent of children with ITP will recover

within a few months of presentation with or without

treatment

70 to 80 percent of children with ITP will recover

Most patients do not have serious bleeding including

those with platelet counts


Pharmacologic therapy

IVIG

Steroids

Anti­ Anti ­Rh(D) Rh(D) (Win Rho)

Splenectomy


Patient follow up

Patient admitted overnight and given IVIG

Platelet count after IVIG increased to 97,000

Discharged with a note for preschool (activity

restriction)

Discharged with a note for preschool (activity

Instructed to avoid aspirin, NSAIDS


Platelet counts

Patient Follow Up

Platelet counts

2,000 (admit June 6, 2008)

97,000 (June 10)

67,000 (June 24)

25,000 (July 11)

70,000 (August 8)

140, 000 (October 14)

472,000 (January 29, 2009)


Questions?

Free YOGA class tonight!

6:15pm, Third floor LPCH in the

physical therapy rooms (near the

MD workroom just off 3 South)

Thank you Mark Halpert for

teaching!


References

Steuber, C. Treatment and prognosis of immune

(idiopathic) thrombocytopenic purpura in children.

UpToDate online.

Buchanan, G. Thrombocytopenia During Childhood.

Pediatrics in Review 2005;26:401­

2005;26:401 ­409 409

Chu, Y et all. Itopathic Thrombocytopenic Purpura.

Pediatrics in Review 2000;21:95­ 2000;21:95 ­101 101

Buchanan, G. ITP: How much treatment is enough?

Contemporary Pediatrics 2000;14: 112­ 112 ­121 121

Steuber, C. Treatment and prognosis of immune

Buchanan, G. Thrombocytopenia During Childhood.

Chu, Y et all. Itopathic Thrombocytopenic Purpura.

Buchanan, G. ITP: How much treatment is enough?

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