IMMUNE THROMBOCYTOPENIC PURPURA

ABSTRACT

Immune thrombocytopenic purpura (ITP), also known as idiopathic thrombocytopenic purpura is thought to be due to autoimmune disease. An abnormal autoantibody (immunoglobulin G) binds to the platelet membrane, causing removal by the spleen. If left untreated it could lead to bleeding and possibly death (Psaila, 2007). It is a rare pathology with an incidence of 3.25 cases per 100,000. Typically occurring in middle-aged women but risk increases with age. Physical exam is usually normal but pectechaie may be present on lower extremities and/or mucosal membranes. A complete blood count and peripheral smear show isolated thrombocytopenia. Diagnosis is made by exclusion of secondary causes of ITP. Treatment is based on platelet count and presence of bleeding symptoms. Therapies usually involve combinations of steroids, IV immunoglobin, platelet transfusion and splenectomy (Cines, 2009).

CLINICAL PRESENTATION

CC: Bruising easily x 3 months HPI: 21yo female states she had been bruising very easily

for the past 3 months. Noticed small purple/red bruises located mostly on the arms and legs. The bruising frequency has progressively increased. Minor bumps/pressure form bruises when they had not before. Does not notice anything that alleviates or any other associated sx. Up until this point she had refused to have blood work done as she “hates needles” but now is willing.

ROS: Denies fever, chills, fatigue, anorexia, weight loss, GI bleeding,

lymphadenopathy, recurrent infxns, dyspnea, rashes, changes in vision, N/V/D, arthralgia, LE edema and she denied domestic abuse three times during the visit.

CLINICAL PRESENTATION CONT…

PMHx: Asthma, anxiety, bipolar disorder, high risk sexual behavior,

depression, allergic rhinitis, low-grade squamous intraepithelial lesion

FHx: Maternal grandmother - DM II Maternal Grandfather - CAD Paternal grandmother - HTN

SHx: Single mother, currently unemployed. Tobacco 1 PPD x 5 yrs and drinks 1-2 beers/mo. Denies IV drug use.

Meds: Mirena 20 ug/24h IUD Loratadine 10mg PO qD

Allergies: No Known Drug Allergies

PHYSICAL EXAM Vitals: T: 98.4⁰F, BP:100/64, HR:96, R: 16, Ht: 5' 2", Wt: 159 lb 6.4 oz, BMI: 29.15 (kg/m^2). GEN: 21yo caucasian female who is alert, healthy, in no distress, well nourished and well

developed. SKIN: Bruises of the arms, legs and both breasts in varying stages of healing.

+Black/blue bruises of right upper arm; +Yellow bruise of posterior upper right arm; +Green bruise of anterior left shin. Breasts: +yellow bruising of breasts bilaterally.

HEAD: Normocephalic, equal hair distribution, face is symmetric. TMJ is nontender & without crepitus.

EYES: PERRLA, EOM intact, Conjunctiva are pink and non-injected, sclera clear. EARS: Tragus and helix is non-tender on palpation. Right and left tympanic membranes are pearly

gray, cone of light and handle of malleus visualized. No discharge or bleeding of canals noted. NOSE: Bridge and naris patent without gross deformities. No pressure in maxillary or frontal

sinuses. Mucosa membranes are pink and moist. OROPHARYNX: Buccal mucosa is pink and moist, most teeth are present with very poor dentition, gums healthy without bleeding. No external ulcers or fissures of lips, tonsillar enlargement. NECK: supple with no lymphadenopathy or JVD LYMPHATICS: no palpable lymphadenopathy LUNGS: clear to auscultation. HEART: regular rate & rhythm. No murmurs, gallops or rubs. ABDOMEN: Active BS, abdomen soft, non-tender and no masses or organomegaly Musculoskeletal: No joint edema, erythema, atrophy or boney deformity. Full active ROM of upper and lower extremities without crepitus. NEURO: Gait normal, Sensory/Motor grossly intact.

DIFERRENTIAL DIAGNOSIS

Immune thrombocytopenic purpura

Systemic lupus erythematosus

Chronic lymphocytic leukemia

Non-hodgkins lymphoma Ethanol toxicity HIV or H. pylori infection Heparin Blood transfusion Sepsis Hemolytic uremic syndrome

Thrombotic thrombocytopenic purpura

Myelodysplastic syndromes Disseminated intravascular

coagulation (DIC) Pseudothrombocytopenia Gestational

thrombocytopenia/ Severe pre-eclampsia

Drug-induced (quinine or sulfamides)

Hypersplenism

LABORATORY & OTHER DIAGNOSTIC FINDINGS

Initial Labs: ANA, Platlet Ab direct, Platlet Ab indirect, Factor VIII, aPTT, PT/INR, CBC Platelets 6,000 K/uL

ASSESSMENT Immune Thrombocytopenic Purpura Initial Treatment:

prednisone 60mg PO qD Adults: 1-2mg/kg/day, taper dose gradually once

platelet count has reached plateau. Stop anti-platelets (i.e. aspirin) Refer to Hematology

CLINICAL COURSE

25-Ja

n

3-Fe

b

12-F

eb

21-F

eb

2-Mar

11-M

ar

20-M

ar

29-M

ar

7-Apr

16-A

pr

25-A

pr

4-May

13-M

ay

22-M

ay

31-M

ay9-

Jun

18-Ju

n

27-Ju

n6-

Jul

15-Ju

l

24-Ju

l

2-Aug

11-A

ug

20-A

ug

29-A

ug

7-Se

p

16-S

ep

25-S

ep4-

Oct0

50,000

100,000

150,000

200,000

250,000

300,000

350,000

400,000

Chart Title

Time

Pla

tlet

Count

CLINICAL COURSE

Treatment Now: Laparoscopic Splenectomy BUT FIRST!! vaccinate for encapsulated

organisms at least 2 weeks prior to splenectomy.

influenza, meningococcal, pneumococcal and hib

Imaging : Abdominal CT w/ no IV contrast IMPRESSION:

1. Unremarkable appearance of the spleen.2. Cholelithiasis.

PATHOPHYSIOLOGY

Abnormal autoantibody (immunoglobulin G) binds to the platelet membrane, causing removal by the spleen. Although ITP is mostly asymptomatic if left untreated it could lead to bleeding and possibly death.

Low platelet count <100,000/mm3

Epidemiology: Typically affects middle-aged women. Children: 2-5 years; Adults: 20-50 years. >>>> Risk increases with age <<<< Children: males = females; Adults: females>males (3:1) 40% of diagnoses are children <10 years of age Spontaneous remission: >80% in children and <20% in adults

2 clinical presentations: Acute ITP: Preceding viral infection; resolves spontaneously within 3 mo (90% of

pediatrics). Chronic ITP: insidious onset of easy bruising/minor bleeding over a few months, no

antecedent infection, and duration >6 months (Adults). Intracranial hemorrhage is the most frequent cause of death, occurring

in 1-2% of patients diagnosed. Spontaneous bleeding: platelet count < 20,000/mm3 (20x109/L)

Physical exam: Often normal but pectechaie may be present on lower extremities and/or mucosal membranes.

REFERENCES

Cines, D.B. (2009). The ITP syndrome: pathogenic and clinical diversity. Blood, 26(113), 6511-21.

Psaila, B. (2007). Immune thrombocytic purpura. Hematol Oncol Clin North America, 21(4), 743-59.

Scully, C. (2009). Aspects of human disease. Dental Update, 7(36), 449.