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<strong>VISUAL</strong> <strong>DIAGNOSIS</strong>


A “ spidery ” rash<br />

Lindsay Jackson, MD<br />

October 14, 2009


Case of a “spidery “ spidery” ” rash<br />

3 year old male develops a spidery rash over<br />

the past week on his arms and legs<br />

3 year old male develops a spidery rash over<br />

Bruising noted over lower extremities<br />

Dried blood noted at ears, nose<br />

“Blood “ Blood blister” blister ” on lower lip


Purpura


The “spidery “ spidery” ” rash


Exam<br />

Growth parameters and vital signs normal<br />

Well appearing<br />

HEENT: ecchymosis over left eye, crusted<br />

blood in nares, palatal petechiae<br />

HEENT: ecchymosis over left eye, crusted<br />

Abdomen: no hepatosplenomegaly<br />

Skin: petechiae on arms, back, legs. Pretibial<br />

bruising<br />

Skin: petechiae on arms, back, legs. Pretibial


Differential Diagnosis<br />

What causes thrombocytopenia (platelet count<br />

below 150,000) in children?<br />

What causes thrombocytopenia (platelet count<br />

Idiopathic thrombocytopenic purpura<br />

Drug induced thrombocytopenia (heparin)<br />

TTP<br />

Sepsis/DIC<br />

Congenital<br />

TAR (Thrombocytopenia, absent radii)<br />

Kasabach­ Kasabach ­Merritt Merritt<br />

Wiskott­ Wiskott ­Aldrich Aldrich


Labs<br />

WBC 7.3 (29% neutrophils, 63% lymphocytes,<br />

6% monocytes, 2% eosinophils)<br />

WBC 7.3 (29% neutrophils, 63% lymphocytes,<br />

Hemoglobin 11.9 with Hematocrit 34.4<br />

Platelet count 2000<br />

Chemistry panel<br />

Chemistry panel<br />

Na 132, K 5.6 (Hemolyzed) and LDH 473<br />

Liver function studies normal


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

Lazarchick, J. ASH Image Bank 2001;2001:100177<br />

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


Idiopathic Thrombocytopenic<br />

Objectives<br />

Purpura (ITP)<br />

Objectives<br />

Know function and life span of platelets<br />

Describe the usual presentation and laboratory<br />

tests in the child with ITP<br />

Describe the usual presentation and laboratory<br />

Review the treatment options<br />

Know the usual prognosis


Normal peripheral smear<br />

Normal platelets are one fifth the<br />

diameter of erythrocytes.<br />

They survive in the body for 9 to 10<br />

days


Idiopathic Thrombocytopenic<br />

Purpura (ITP)<br />

Estimated to be one of the most common<br />

acquired bleeding disorders encountered by<br />

pediatricians<br />

Estimated to be one of the most common<br />

3 to 8 cases per 100,000 children per year<br />

3 to 8 cases per 100,000 children per year<br />

A general pediatrician can care for most<br />

children with ITP<br />

A general pediatrician can care for most


ITP<br />

Autoantibodies (usually IgG) are directed against<br />

platelet membrane antigens<br />

Autoantibodies (usually IgG) are directed against<br />

Antibody­ Antibody ­coated coated platelets have a shortened half­ half ­life life<br />

because of accelerated clearance by tissue<br />

macrophages in the spleen and other portions of the<br />

reticuloendothelial system.<br />

The net effect is a decrease in the platelet count.


IgG coated Platelets


Presentation<br />

Most common in ages 2 to 5<br />

Characterized by the sudden appearance of<br />

bruising/bleeding/petechiae in an otherwise<br />

healthy child<br />

Characterized by the sudden appearance of<br />

Association with prior infection<br />

Association with MMR vaccination


Petechiae in ITP


Pertinent Negatives<br />

No systemic symptoms<br />

Absence of lymphadenopathy and<br />

hepatosplenomegaly on exam<br />

No evidence of hemolysis<br />

Absence of lymphadenopathy and<br />

No evidence of hemolysis


Laboratory findings<br />

Platelet count typically below 30,000<br />

15% of patients will have mild anemia due to<br />

bleeding<br />

Essential to review peripheral smear<br />

15% of patients will have mild anemia due to<br />

Essential to review peripheral smear<br />

Large platelets


Leukemia<br />

Leukemia<br />

What do parents fear?<br />

In one study of 2239 patients with newly<br />

diagnosed ALL, NONE exhibited isolated<br />

thrombocytopenia<br />

Bone marrow examination not necessary in routine<br />

cases<br />

In one study of 2239 patients with newly<br />

Bone marrow examination not necessary in routine<br />

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

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

Pediatrics 1986;77:49


Chronic ITP<br />

Thrombocytopenia lasting longer than 6<br />

months<br />

Thrombocytopenia lasting longer than 6<br />

Thyroid studies<br />

Thyroid studies<br />

ANA<br />

Coombs test<br />

HIV/CMV/EBV<br />

Immunodeficiency


Pharmacologic Therapy<br />

70 to 80 percent of children with ITP will recover<br />

within a few months of presentation with or without<br />

treatment<br />

70 to 80 percent of children with ITP will recover<br />

Most patients do not have serious bleeding including<br />

those with platelet counts


Pharmacologic therapy<br />

IVIG<br />

Steroids<br />

Anti­ Anti ­Rh(D) Rh(D) (Win Rho)<br />

Splenectomy


Patient follow up<br />

Patient admitted overnight and given IVIG<br />

Platelet count after IVIG increased to 97,000<br />

Discharged with a note for preschool (activity<br />

restriction)<br />

Discharged with a note for preschool (activity<br />

Instructed to avoid aspirin, NSAIDS


Platelet counts<br />

Patient Follow Up<br />

Platelet counts<br />

2,000 (admit June 6, 2008)<br />

97,000 (June 10)<br />

67,000 (June 24)<br />

25,000 (July 11)<br />

70,000 (August 8)<br />

140, 000 (October 14)<br />

472,000 (January 29, 2009)


Questions?<br />

Free YOGA class tonight!<br />

6:15pm, Third floor LPCH in the<br />

physical therapy rooms (near the<br />

MD workroom just off 3 South)<br />

Thank you Mark Halpert for<br />

teaching!


References<br />

Steuber, C. Treatment and prognosis of immune<br />

(idiopathic) thrombocytopenic purpura in children.<br />

UpToDate online.<br />

Buchanan, G. Thrombocytopenia During Childhood.<br />

Pediatrics in Review 2005;26:401­<br />

2005;26:401 ­409 409<br />

Chu, Y et all. Itopathic Thrombocytopenic Purpura.<br />

Pediatrics in Review 2000;21:95­ 2000;21:95 ­101 101<br />

Buchanan, G. ITP: How much treatment is enough?<br />

Contemporary Pediatrics 2000;14: 112­ 112 ­121 121<br />

Steuber, C. Treatment and prognosis of immune<br />

Buchanan, G. Thrombocytopenia During Childhood.<br />

Chu, Y et all. Itopathic Thrombocytopenic Purpura.<br />

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

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