A Case Presentation - Pedia

A Case Presentation:

Clues from the BruiseBy: PGI Ryan Abutazil

Objectives:

1. To be able to present an actual case of immune thrombocytopenic purpura

2. To be able to discuss the pathologic process of the disease

3. To present therapeutic options for ITP4. To be able to present updates on ITP

Clinical Historyo J.P.H.o 2 years oldo Maleo Islamo Manlubal, R.T. Lim, Zamboanga

Sibugay

Chief Complaint: spontaneous bruise formation

1. Twelve days PTA (+) raised non-erythematous, non-tender, non-pruritic skin lesions (+) fever (+) decrease in appetite (+) weight loss

Clinical History

2. Nine days PTA(+) spontaneous formation

of P5 coin-size bruises, non-tender

(+) pin point petechial rashes

(-) fever(-) bleeding diathesis

Clinical History

(+) consult at Ipil Provincial Hospital, was given HEMARATE and was referred to this institution.

3. Three days PTA(+) consult at the OPD Dept. of

ZCMC(+) laboratory work up was

requested

Clinical History

CBC with platelet

Clinical HistoryHct 0.35

WBC 5.2

Diff. Count

Segmenters

0.29

Lymphocytes

0.60

Eosinophils

0.08

Monocytes

0.03

Platelet Count

108

SMEARS SHOW NORMOCHROMIC RBC’s WITH MODERATE ANISOPOIKILOCYTOSIS. THE CELLS IN THE WBC SERIES ARE MATURED. PLATELETS ARE INADEQUATE IN NUMBERS

Peripheral Blood Smear

4. On the night PTA(+) had an onset of

epistaxis, spontaneously resolved

(-) meds taken, (-) vomiting, (-) abdominal pain (-) cyanosis,

(-) pallor (-) jaundice

Clinical History

(-) allergies to food and medication(-) previous hospitalization(-) past exposure to radiation or chemicals(-) Heredofamilial diseases such as leukemia or anemia or other blood dyscrasia.

Clinical History

Born to a G1P1 mother, delivered institutionally full term via NSVD; compliant to prenatal check up(-) maternal illness(+) immunization(+) Bottlefed since birth (NESTOGEN)

Clinical History

At the ER, patient was seen -awake-ambulatory -irritable -NICRD

Vital signs: BP- 90/60mmHg Temp- 36.2 PR- 116 RR- 35

Physical Exam

Weight: 11 kg. Height: 82 cm.

Physical Exam

Skin: Ecchymoses at the left lateral portion of the trunk and anterior areas of both legs below the knees; Petechiae on the areas of the forehead, left cheek and lower lips (-) pallor (-) jaundice (-) cyanosis

EENT: Pink Palpebral Conjunctiva, Anicteric sclerae, midline nasal septum with no signs of

trauma or bleeding, moist oral mucosa,

(-) lymphadenopathy,

Physical Exam

Chest and Lungs: ECE, (-) IC & subcostal retractions, (-) chest indrawings, (-) crackles, (-) rales, (-) adventitous breathe sounds

Physical Exam

Heart: adynamic precordium with normal rate and regular rhythm, (-) murmurs

Physical Exam

Abdomen: flat, nondistended, normoactive bowel sounds, soft, non tender, no palpable mass, no organomegaly

Extremities: good, equally strong pulses with good CRT

Physical Exam

Immune Thrombocytopenic Purpura (ITP)with mild symptoms

Admitting Impression

History:- 2 years old- male- a preceding infection (raised, non-erythematous, non-tender skin lesions and fever)- abrupt onset of spontaneous bruises on the trunk and legs and pin point rashes on the face- CBC results with PBS- Epistaxis

Basis for Diagnosis

Physical Exam:- Skin: Ecchymoses at the left lateral portion of the trunk and anterior areas of both legs below the knees; Petechiae on the areas of the forehead, left cheek and lower lips - no lymphadenopathy- no organomegaly

Basis for Diagnosis

Differential Diagnosis

Thrombotic Thrombocytopenic

purpura

Hemolytic Uremic

Syndrome

Leukemia

spontaneous bruising,

petechiae, weight loss

(-) lymphadenopathy (-) splenomegaly

(-) bone tenderness(-) WBC derangements


petechiae, weight loss

(-) signs of hypertension(-) renal impairement

spontaneous bruising, petechiae,

weight loss

(-) Neurologic manifestations include alteration in mental status, seizures, hemiplegia, paresthesias, visual

RULED IN RULED OUT


Wiskott-Aldrich Syndrome

Fanconi’s anemia

Male, unusual bruising, occurs

primarily in children

Absence of atopic symptoms like

eczema

(-) pancytopenia in CBC

(-) congenital birth defects


petechiae, epistaxis, purpura

RULED IN RULED OUT


Drug-induced thrombocytopenia

Aplastic anemia

Absence of history of drug intake





Other than thrombocytopenia,

CBC result is normal

Thrombocytopenia with absent

radii



Age of onset is 1st week of life;

(-) gross physical abnormalities

RULED IN RULED OUT

Course in the Ward:May 9, 201112:00PM

(+) awake, comfortable(-) afebrile(+) ecchymosses(+) petechiae(-) organomegaly(+) good pulses

Acute ITP with mild symptoms

Admitted to Ward 8 (ISO) Secure consent TPR q 4 hours DAT Insert IV heplock Lab work-up 1. CBC, platelet, Blood typing - done 2. Peripheral Blood Smear - done For BMA tomorrow in the morning Secure consent For repeat CBC, Platelet, and PBS tomorrow morning I and O q shift and record Refer for any untoward event

May 9, 201110:00PM

NPO post breakfast at 6:00 AM Start D 5.3 NaCl 500 cc at MR once on NPO Refer accordingly

1st Hospital DayDate S O A P

May 9, 2011 Comfortable Not irritable No bleeding

episodes Good

appetite Good activity

Temp: 37.0 PR: 105 RR: 27 Awake (+) ecchymoses

and petechaie PPC Nondistended

abdomen (-) organomegaly Good pulses


TPR q 4 hours DAT Lab work-up 1. CBC, platelet, Blood typing 2. Peripheral Blood Smear For BMA

tomorrow in the morning

Secure consent

For repeat CBC, Platelet, and PBS tomorrow morning

I and O q shift and record

2nd Hospital DayDate S O A P


episodes Good

appetite Good

activity

Afebrile with normal PR and RR

Awake, playing (+) ecchymoses

and petechaie PPC CBS, (-) crackles Nondistended

abdomen (-) organomegaly Good pulses (-) cyanosis


For BMA: secure materials and consent from watcher

Monitor V/S without fail

S/P BMA: maintain flat on bed for 6 hours refer if site is bleeding continuously Start

Prednisone 10mg per 5mL, 10 mL once a day per orem.

Rpt CBC Plt:Hgb 123Hct 0.39RBC 5.36WBC 13.0 Seg 0.33 Lymph 0.57 Mono 0.10Platelet 38MCV 72.4MCH 22.9MCHC 31.7

PBSRBCs are moderately hypochromic; WBC series are unaltered; Platelets are diminished

3rd Hospital DayDate S O A P

May 11, 2011 New areas with petechial rashes on the face and neck

No epistaxis

No melena

Comfortable Asleep Temp: 37.3 PR 111 RR 25 (+) petechial

rashes & ecchymoses

Ppc, (-) epistaxis, (-) gum bleeding

(-) lymphadenopathy

ECE, clear breath sounds with no crackles or wheezes

NRRR (-) murmur

Same abdominal findings

Good pulses, (-) cyanosis


IVF to consume then terminate

For repeat CBC, Platelet

Continue V/S monitoring

Rpt CBC Plt:Hgb 123Hct 0.37RBC 5.36WBC 8.0 Seg 0.64 Lymph 0.35 Mono 0.01Platelet 98

PBSNone

4th Hospital DayDate S O A P


episodes Good

appetite Good activity

Temp: 36.9 PR: 98 RR: 21 Awake, well-

nourished (+) ecchymoses

and petechaie PPC Nondistended

abdomen (-) organomegaly Good pulses


Terminate IVF MGH Continue

Prednisone Oral medication at home

Ff up scheduled

Final Diagnosis:


What is ITP?

Immune

Thrombocytopenic

Purpura

What happens in normal hemostasis?

What Occurs in ITP?

Increased peripheral destruction of platelets

Humoral Immunity is involved primarily

a. production of antibodies that identifies the platelet surface proteins as foreign.

b. These antibodies work in two ways: OPSONIZATION, a process where the antibody coats the platelet for optimum recognition and subsequent phagocytosis by macrophages and polymorphonuclear neutrophils, and COMPLEMENT PATHWAY ACTIVATION, which when activated destroys platelet wall integrity, resulting in cell lysis.

What Occurs in ITP?

Eventually, immune destruction of platelets is more than its production, which results to depletion of platelets peripherally below 100,000.

Depleted platelets could not hold a solid platelet plug to prevent bleeding at the site of vascular injury.

The problem is primarily on platelet destruction, and not on platelet production.

Acute ITP Chronic ITP

Children > adults

(2-6 years old)

Adults mainly(30-50 years old)

Females = Males Females > males

Self-limiting Chronic in most

Post viral No preceding illness

Lasts within 6 months

Lasts >6 months

Spontaneous remission in

85%

Spontaneous remissions 2%

Clinical Manifestations


Chronic InfantileITP

Chronic Childhood ITP

Age 4-24 months > 24 months

Male/Female ratio 3:1 1:3

Onset Abrupt Insidious

Preceding Infection

Unusual Frequent

Platelet Count <20,000 40,000-80,000

Response to Therapy

poor Temporary

Incidence of Chronicity

30% 10-15%


Abrupt onset of bruises and petechial rashes on an apparently well child

Mucosal membrane bleeding, e.g. epistaxis or gum bleeding episodes

A history of a viral illness 1 to 3 weeks prior to the onset of the rashes

Gross hematuria or gastrointestinal bleeding

There is normal spleen and liver size

Diagnosis

The diagnosis of Immune Thrombocytopenic Purpura is established once you have excluded other possible factors for the thrombocytopenia, such as leukemia, myelodysplasia, aplastic anemia, or adverse drug reactions.

CBC results will show no abnormalities in the RBC and WBC counts, unless prolonged bleeding has occurred.

Peripheral Blood Smear of ITP is non-specific since other thrombolytic thrombocytopenias also show larger platelets with increased platelet diameters.

Diagnosis

Bone Marrow Aspiration:

- the presence of megakaryocytes in normal or increased numbers confirms that the thrombocytopenia is thrombolytic and not a problem of production

- absence of leukemic infiltrates rules out malignancy as the cause of the platelet decrease.

- Eosinophils may be abundant in BMA of ITP patients

Patient ManagementWhen do we treat?

a. Treatment is indicated if there has been prolonged mucosal bleed such as epistaxis or gum bleeding

b. If possible intracranial bleed is imminentc. Still, the physician’s clinical judgement is of

utmost importance in deciding whether to treat a child with ITP, since oral corticosteroids also carry several side effects that would affect the patient in the long term.

Pharmacological Patient Management

Corticosteroids : oral prednisone at a dose of 1 to 2 mg per kg per day (60 to 80 mg maximum). In giving this, vascular stability is increased and the endothelial abnormalities are resolved. Side effects: growth retardation, osteoporosis, cataracts, hypertension, acne, psychosis and cushingoid facie.

1. Prolongs platelet survival from splenic destruction by inhibiting phagocytosis

2. Improves platelet preservation and economy by increasing capillary resistance3. Suppresses immune response against platelet

Indication:

1.Excessive bleeding from the mucous membrane

2.Extensive purpuric lesions especially on the head and neck

3.Thrombocytopenia of more than 5 weeks.

4.Recurrent Thrombocytopenia5.Platelet count of less than

30,000


High dose IV gammaglobulin1. blocks Fc Receptors of the RES2. Protection of platelets and or megakaryocytes from platelet antibody3. Also acts as an antiviral.

Indications: 1. infants less than 2 years old who

are refractory to steroid therapy

2. This is a valid alternative for steroid therapy

Beneficial Effects: 1. it actually decreases

bleeding episodes compared to steroid treatment

2. increases platelet production more significantly than steroid therapy

Toxic Effects:1. Fever and chills in 1-3% of

patients2. Anaphylaxis in IgA-deficient

patients.


Anti-Rh D TherapyInfusion of Anti-Rh D in patients with

Rh positive will mount an immune response againts the patient’s RBC’s which will result to a transient hemolytic anemia. Coincident to the clearance of the antibody-coated RBC’s is the increase in the platelet survival in the patients with ITP. This is due to the blocking of the Fc receptors of the reticuloendothelial system.


VinblastineDanazolCyclophosphamideAzathioprineCyclosporineAlpha- interferon

Platelet transfusion- (+) active neurologic signs

Splenectomy- for severe acute ITP with acute life threatening bleeding, with a non-responsive medical treatment

Laparoscopic splenectomy for immune thrombocytopenic purpura at a teaching institution.Zheng CX, Zheng D, Chen LH, Yu JF, Wu ZM.SourceDepartment of General Surgery, the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong 510080, China (Email: [email protected]).

BACKGROUND:High anatomic location, fragility, and generous blood supply of the spleen makes laparoscopic splenectomy (LS) difficult to master, and few patients need splenectomy for benign disorders. The aim of this research was to assess operative outcomes and hematological results of a large series of patients treated with LS for chronic immune thrombocytopenic purpura (ITP) and to determine which clinical variables predict favorable hematological outcome.

http://www.ncbi.nlm.nih.gov/pubmed?term=%22Zheng%20CX%22%5BAuthor%5D

http://www.ncbi.nlm.nih.gov/pubmed?term=%22Zheng%20CX%22%5BAuthor%5D

http://www.ncbi.nlm.nih.gov/pubmed?term=%22Zheng%20D%22%5BAuthor%5D

http://www.ncbi.nlm.nih.gov/pubmed?term=%22Zheng%20D%22%5BAuthor%5D

http://www.ncbi.nlm.nih.gov/pubmed?term=%22Chen%20LH%22%5BAuthor%5D

http://www.ncbi.nlm.nih.gov/pubmed?term=%22Yu%20JF%22%5BAuthor%5D

http://www.ncbi.nlm.nih.gov/pubmed?term=%22Wu%20ZM%22%5BAuthor%5D

METHODS:LS was successfully performed for 154 patients with chronic ITP from September 1999 to April 2009 at the First Affiliated Hospital of Sun Yat-sen University. Operative outcomes were assessed retrospectively. Long-term follow-up data were obtained from outpatient medical records and phone interviews.

Clinical and laboratory variables (including gender, age, disease duration before surgery, previous response to steroids, preoperative platelet count, and postoperative peak platelet count) were evaluated by univariate analysis to identify potential predictors of hematological outcome.

Multivariate Logistic regression model was used to determine independent predictors of hematological outcome.

RESULTS:One patient died from subphrenic abscess and postoperative sepsis. The overall major morbidity rate was 8.4%. None of the patients required a second surgery for complications. Of the 127 patients available for a mean follow-up of 43.6 months (range 9 - 114 months), the overall initial response (i.e., at two months after LS) and long-term response to LS were achieved in 89.0% and 80.3%, respectively. Five patients (3.9%) developed pneumonia 3 - 35 months after LS.

Univariate analysis showed a significant difference in mean age between responders (29.1 years) and nonresponders (38.8 years; P < 0.05). Patients who responded to steroid therapy had better hematological outcome than those who did not respond (P < 0.05). Compared to nonresponders, responders to LS had a significantly higher postoperative peak platelet count (404 × 10(9)/L versus 213 × 10(9)/L, P < 0.001).

CONCLUSIONS:LS is a safe and effective treatment for chronic ITP. Postoperative peak platelet count may serve as a major predictor of long-term response.

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