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![• No risk, with platelet count of 50 to 150x109/L
• Rarely causes bleeding even with trauma, with platelet count of 30 – 50 x109/L.
• Bleeding with significant trauma but is unusual with normal day to day activity, with
platelet count of 10 – 30 x109/L (many patients are asymptomatic).
• Spontaneous bruising or bleeding may occur with platelet count of <10 x109/L
(many patients are asymptomatic).
• Critical spontaneous bleeding (eg, atraumatic intracranial hemorrhage [ICH]) may
occur with platelet count of <5 x109/L.
A p p r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a
Bleeding risk: (based on degree of Thrombocytopenia)
Bleeding risk is also dependent on whether other parts of the hemostatic process are involved e.g., coagulation
factor abnormalities in liver disease.](https://image.slidesharecdn.com/approachtopediatricpatientwiththrombocytopenia-240127182040-4bcc48c7/75/Approach-to-Pediatric-patient-with-thrombocytopenia-pptx-8-2048.jpg)
Uploaded byMustafa selim
Approach to Pediatric patient with thrombocytopenia.pptx
This document provides an overview of evaluating pediatric patients with thrombocytopenia. It defines degrees of thrombocytopenia and associated bleeding risks. The evaluation involves obtaining a detailed history, clinical assessment, and initial laboratory tests including a complete blood count and peripheral blood smear. The history aims to identify potential etiologies such as infections, drugs, recent illness or procedures. The clinical exam evaluates for signs of bleeding and possible underlying conditions. The blood tests can help determine if thrombocytopenia is real or pseudo, and provide clues to possible causes through findings on the peripheral smear. Further testing may then be guided by these initial findings.
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Approach to Pediatric patient with thrombocytopenia.pptx
- 1. Course Benign Hematology Mustafa Selim,MD Lecturer of Pediatric Oncology, NCI, Cairo University – Consultant of Pediatric Oncology, CCHE - 57357 Egypt Clinical fellowship program trainer (CCHE-57357/Dana-Farber Cancer Institute pediatric Oncology Fellowship)
- 2. Objectives A p pr o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Definition - degree - bleeding risk? 1 2 3 Clinical, laboratory and genomic approach? Key features of the clinical & lab evaluation? Ttreatment, follow up 4
- 3. Blood ismade of two major components: plasma + cells. CBC = complete blood count→ is a group of tests that evaluate the cells that circulate in blood, including RBCs, WBCs, and PLTs. Collected in purple tube (EDTA) Unit volume: per cubic millimeter (mm3) = one microliter = µL WHAT cbc?
- 4. Automated counting Erythrocytes Leukocytes Thrombocytes Count4-5.5 x 106/µL 4-11 x 106/µL 150 - 450 x 109/µL Size 7 microns 8 – 20 microns 3 – 4 microns Life span 120 days 7 – 10 days • Coulter principle: electrical impedance: resistance or change in current when cell passes between electrodes in NaCL solution • Flow cytometry: uses lasers to measure both forward and side scatter • Forward scatter measures size. • Side scatter measures granularity
- 5. What is thesuccess?
- 7. Thrombocytopenia degree (MehmetAli 2012) • The normal platelet count ranges from 150 to 450x109/L. • Thrombocytopenia, defined as a platelet count of < 150x109/L. • Mild Thrombocytopenia, defined as a platelet count of >100 x109/L. • Moderate Thrombocytopenia, defined as a platelet count of 51 to 100 x109/L. • Severe Thrombocytopenia, defined as a platelet count of 21 to 50 x109/L. • Very severe Thrombocytopenia, defined as a platelet count of < 20x109/L. A p p r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Definition
- 8. • No risk,with platelet count of 50 to 150x109/L • Rarely causes bleeding even with trauma, with platelet count of 30 – 50 x109/L. • Bleeding with significant trauma but is unusual with normal day to day activity, with platelet count of 10 – 30 x109/L (many patients are asymptomatic). • Spontaneous bruising or bleeding may occur with platelet count of <10 x109/L (many patients are asymptomatic). • Critical spontaneous bleeding (eg, atraumatic intracranial hemorrhage [ICH]) may occur with platelet count of <5 x109/L. A p p r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Bleeding risk: (based on degree of Thrombocytopenia) Bleeding risk is also dependent on whether other parts of the hemostatic process are involved e.g., coagulation factor abnormalities in liver disease.
- 9. A p pr o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Bleeding risk: (based on degree of Thrombocytopenia) Relationship between major bleeding and platelet count. Adapted from Slichter SJ. Relationship between platelet count and bleeding risk in thrombocytopenic patients. Transfus Med Rev. 2004;18:153–167
- 10. Thrombocytopenia can be Types Idiopathic Acquired Sequestration/other Seconday eg,Drugs, infections A p p r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Inherited Normal - Small - Large platlet size Increased platelet consumption Decreased platelet production
- 11. A p pr o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Aetiology Immune-mediated 1. Immune thrombocytopenic purpura 2. Drug-induced thrombocytopenia 3. Autoimmune syndromes (e.g., antiphospholipid syndrome, SLE, Evans’ syndrome, sarcoidosis, Autoimmune lymphoproliferative syndrome) 4. Alloimmune destruction (e.g., posttransfusion, neonatal, post transplantation) Non-Immune-mediated 1. Infections (e.g., CMV, EBV, HCV, HIV, mumps, parvovirus B19, rickettsia, rubella, varicella- zoster virus) 2. Mechanical destruction (aortic valve) 3. TTP/HUS, DIC Increased platelet consumption Decreased platelet production Sequestration/other 1) Infections (e.g., CMV, EBV, HCV, HIV, mumps, parvovirus B19, rickettsia, rubella, varicella- zoster virus) 2) Nutritional deficiencies (vit B12 and folate) 3) Congenital thrombocytopenia (e.g., Alport syndrome, Bernard Soulier syndrome, Fanconi anemia, platelet-type or pseudo– von Willebrand disease, Wiskott-Aldrich syndrome) 4) BM failure (e.g., aplastic anemia, PNH, Schwachman-Diamond syndrome) 5) BM suppression (e.g., from medication, chemotherapy, or irradiation) 6) Myelodysplastic syndrome 7) Neoplastic marrow infiltration 1) Pseudo thrombocytopenia 2) Dilutional thrombocytopenia (e.g., hemorrhage, excessive crystalloid infusion) 3) Hypersplenism 4) Liver disease (e.g., cirrhosis, fibrosis, portal hypertension) 5) Burn 6) Hypothermia 7) Pulmonary emboli 8) Pulmonary hypertension 9) Gestational thrombocytopenia
- 12. A p pr o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Aetiology of platelet disorders Acquired Inherited WAS, Wiskott-Aldrich syndrome; XLT, X-linked thrombocytopenia; CAMT, congenital amegakaryocytic thrombocytopenia; RUSAT, radioulnar synostosis with amegakaryocytic thrombocytopenia; TAR, thrombocytopenia-absent radius; QPD, Quebec platelet disorder; XLTDA, X-linked thrombocytopenia with or without dyserythropoietic anemia; MYH9, MYH9-related disorders; BSS, Bernard-Soulier syndrome; vWD, von Willebrand disease; GPS, gray platelet syndrome; JS, Jacobsen syndrome; PTS, Paris-Trousseau syndrome; GT, Glanzmann thrombasthenia; HPS, Hermansky-Pudlak syndrome; CHS, Chediak-Higashi syndrome Shim, Y.J., 2020. Genetic classification and confirmation of inherited platelet disorders: current status in Korea. Clinical and Experimental Pediatrics, 63(3), p.79.
- 13. A p pr o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Acquired Inherited Kirchmaier, C.M. and Pillitteri, D., 2010. Diagnosis and management of inherited platelet disorders. Transfusion Medicine and Hemotherapy, 37(5), pp.237-246. Aetiology of platelet disorders
- 14. A p pr o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Aetiology Common causes of Thrombocytopenia with Clinical Findings and Suggested Treatment Cause Severity Clinical Picture Pseudo thrombocytopenia Factitious Asymptomatic; in vitro agglutination of platelets Infections Mild to moderate Viral: prodrome or asymptomatic; known viruses include CMV, EBV, HCV, HBV, HIV, parvovirus B19, varicella-zoster virusRickettsial: Lyme disease, Rocky Mountain spotted fever, ehrlichiosis; tick- borne illnesses present with fever, headache, malaise, arthralgias, and rash ITP Moderate to severe Acute, self-limiting disorder, isolated thrombocytopenia in health child with or without bleeding manifestations that can resolves spontaneously. Drug-induced hrombocytopenia Moderate to severe Can range from asymptomatic to evidence of clinical bleeding DIC Severe Acute illness: bleeding, acute renal failure, hepatic and respiratory dysfunction, and shock are common clinical manifestations. Secondary to other comorbid conditions, such as sepsis, trauma, burns, or malignancy Congenital thrombocytopenia Mild to moderate Long history of abnormal platelet counts or family history of thrombocytopenia Bone marrow suppression by irradiation, chemotherapy, or neoplasia Moderate to severe History of exposure, comorbid preexisting disease, or malignancy. Acute leukemias present with fatigue, weakness, bruising, hepatosplenomegaly
- 15. A p pr o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a suggesting a diagnosis other than ITP
- 16. History Genetic Clinical Lab Approachto a patient with Thrombocytopenia?
- 17. Plat< 150,000 ina child No plat clumps No anemia or pancytopenia
- 18. 1.a History • Bleedingsymptoms: • Ask about easy bruising or petechiae, gingival bleeding, epistaxis, melena, hematemesis, hemoptysis, and hematuria usually only when platelets are < 10 x 109/L. • Ask also about bleeding after vaccination, dental procedures or circumcision and excessive menstrual bleeding. • Recent illness/ Travel: • Night sweats, fever, weight loss, arthralgia, and rashes. • Viruses: (HCV, HIV, EBV, parvo viruses), • Malaria, brucellosis, helicobacter pylori. • Drugs (AA): (one of the most common types of thrombocytopenia in the outpatient setting) • Aspirin, quinine, vancomycin, ampicillin, Trimethoprim/sulfamethoxazole, piperacillin, • Acetaminophen, NSAIDs, heparin, • Hydrochlorothiazide, herbal medications, Cimetidine, • Carbamazepine, Phenytoin, and chemotherapy. A p p r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a
- 19. 1.b History • Recentvaccinations: e.g., MMR, varicella, influenza A (H1N1) • Recent transfusion: Alloimmune destruction, posttransfusion purpura, viral infection • Recent valve replacement: Mechanical destruction • Chronic illness: hypersplenism due to chronic liver disease • Nutritional: e.g., vitamin B12, folic acid and copper deficiency • Family history: e.g., Bruising or bleeding, or low platelets (Congenital thrombocytopenia). • Onset of bleeding: • Acute: Acute infection (primarily viral), ITP, drug induced, TTP/HUS, malignancies (eg, leukemia) • Chronic: congenital syndromes, ITP, liver disease, myelodysplastic syndrome. A p p r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a
- 20. 2. Clinical assessment •Bleeding sites: • Skin (e.g., petechiae, purpura, bruising). • Orifices (e.g., epistaxis, mucosal, gastrointestinal, genitourinary) and joints. • The eyes, fundoscopy (e.g., hemorrhage is suggestive of central nervous system bleeding). • Nb; Dry purpura refers to purpura in the skin; wet purpura refers to purpura in the mucosa. • Lymphadenopathy and/or hepatosplenomegaly: • Generalized lymphadenopathy: Viral infections (CMV, EBV, HIV), SLE, myeloproliferative disorders, lymphoproliferative disorders). • Hepatomegaly: Chronic liver disease, acute leukemias, viral infections (CMV, EBV, HBV, HCV) • Splenomegaly: Autoimmune (SLE, sarcoidosis), hypersplenism, viral infections) • Acute rash: viral infections, rickettsial diseases, SLE. • Joint swelling • Neurological symptoms: TTP • Eye (cataract), nails (dystrophic nails), ear (sensorineural hearing loss), oral (leukoplakia) • Dysmorphic features: syndromic or limb defects, and/or short stature. A p p r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a
- 21. 3.a Laboratory • CBC: Repeat CBC to confirm that thrombocytopenia is real. Pseudo thrombocytopenia: Due to cold-reacting platelet agglutinins or platelet binding to neutrophils The agglutinins are often seen in patients with high immunoglobulin levels or infections Platelet satellitism: platelet binding to neutrophils In vitro platelet clumping results from EDTA inadequately anticoagulated specimen. The platelet count should be repeated by collecting blood in a tube with heparin or sodium citrate. Giant platelets are counted as white blood cells by automated counter→ false thrombocytopenia • Other labs: evaluation should include peripheral blood smear, liver & renal functions, coagulation tests. • A peripheral blood smear: can provide diagnostic information on a variety of disorders and should be obtained during the initial evaluation and evaluated by specialized physician. A p p r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a 1st step→Platelet count & PBS
- 22. 3.a Laboratory A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Microscopic peripheral blood smear Imron, A.M.N. and Fitri, Z.E., 2019, May. A classification of platelets in peripheral blood smear image as an early detection of myeloproliferative syndrome using gray level co- occurence matrix. In Journal of Physics: Conference Series (Vol. 1201, No. 1, p. 012049). IOP Publishing. 1st step→Platelet count & PBS A patient with perinatal cytomegalovirus infection shows an atypical lymphocyte (AL) and a normal, small lymphocyte (SL). Lee, A.C.W., 2018. Isolated thrombocytopenia in childhood: what if it is not immune thrombocytopenia?. Singapore medical journal, 59(7), p.390.
- 23. 3.a Laboratory A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a A patient with neonatal transient abnormal myelopoiesis shows two large sized blasts. The blast on the left side has a blebby cytoplasmic border suggestive of a megakaryoblastic origin 1st step→Platelet count & PBS A patient with May-Hegglin anomaly shows two neutrophils with cytoplasmic basophilic inclusions (Döhle bodies, arrows) along side a giant platelet (GP) that is approximately the size of a red cell. Lee, A.C.W., 2018. Isolated thrombocytopenia in childhood: what if it is not immune thrombocytopenia?. Singapore medical journal, 59(7), p.390.
- 24. 3.a Laboratory Commonfindings on peripheral blood smear and their associated diagnosis A p p r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Smear findings Possible diagnosis Comments Platelet agglutination Pseudo thrombocytopenia Clumping of platelets in patients with EDTA-activated antiplatelet antibodies Giant platelets (> normal 7-11 fl) ITP, Congenital thrombocytopenia Caused by increased platelet turnover or release of immature forms into the circulation Megakaryocyte fragments Myelofibrosis Presence of large platelets Platelet hypo granularity Myelodysplastic syndrome, myelofibrosis Suggests impaired bone marrow synthesis Small platelets Wiskott-Aldrich syndrome Atypical lymphocytosis Viral infection (e.g., EBV, CMV) Abundant cytoplasm Basophilic stippling Thalassemia, chronic alcohol use, lead or metal poisoning Ribosomal precipitate (appears as blue granules) throughout the cytoplasm of the red blood cell Nucleated red blood cells Severe hemolysis, myelofibrosis Immature red blood cells secondary to accelerated erythropoiesis Oval macrocytosis Vitamin B12 and folate deficiencies Suggests impaired bone marrow synthesis Round macrocytosis Myelodysplastic syndrome, myelofibrosis, chronic liver disease Suggests impaired bone marrow synthesis Schistocytosis TTP, HUS, DIC, defective prosthetic heart valve Fragmented erythrocytes Target cells Chronic liver disease, hemoglobinopathies Excess hemoglobin in the center of the red blood cell
- 25. 3.b Laboratory A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a • Platelets have a round or oval shape • Measurment: • Normal 1.5–3 μm in diameter • Large platelets = macrothrombocytes (3–7 μm) -(normally present in health persons 5%), • Giant platelets (>7 - 20 μm) (reaching the size of erythrocytes or lymphocytes) 2nd step→ Platelet size Normal platelet 1.5–3 μm Large platelet 3–7 μm Giant platelet >7 - 20 μm
- 26. 3.b Laboratory A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a • The mean platelet volume (MPV) (n, 7 to 11 fL). • Importance of the MPV: • It is an indication of average platelet size and platelet turn over, • Newly platelets are larger and tend to decrease in size with age in the circulation. • Rapid turnover, the platelets will be larger because of the larger size of newly produced platelets • Marked platelet anisocytosis is a typical morphologic feature of MDS and MPN. 2nd step→ Platelet size Robier, C., 2020. Platelet morphology. Journal of Laboratory Medicine, 44(5), pp.231-239.
- 27. 3.b Laboratory A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Causes of large platelets 2nd step→ Platelet size Acquired causes Inherited causes (usually associated with thrombocytopenia) Robier, C., 2020. Platelet morphology. Journal of Laboratory Medicine, 44(5), pp.231-239.
- 28. 3.b Laboratory A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Causes of small platelets (Inherited & acquired) 2nd step→ Platelet size Robier, C., 2020. Platelet morphology. Journal of Laboratory Medicine, 44(5), pp.231-239. Inherited causes Acquired causes
- 29. 3.c Laboratory A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a • The platelet cytoplasm contains fine azurophilic granules that may appear scattered throughout the cytoplasm or concentrated in the centre of the platelet, which has been termed granulomere • Morphological platelet abnormalities may affect: The granulation (hypogranular/agranular platelets, presence of singular large granula) The shape (irregular to bizzare, cytoplasmatic blebs) Both may occur simultaneously (frequently associated with abnormalities in platelet size) Circulating megakaryocytes, micromegakaryocytes and megakaryoblasts The occurrence of abnormal megakaryocytes, micromegakaryocytes and megakaryoblasts on PB smears is usually restricted to pathological conditions (such as MDS, AML, TAM of Down syndrome, PMF, post polycythaemia or post thrombocythaemia myelofibrosis and CML). 3rd step→Platelet morphology
- 30. 3.c Laboratory A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Causes of hypogranular/agranular platelets Robier, C., 2020. Platelet morphology. Journal of Laboratory Medicine, 44(5), pp.231-239. 3rd step→Platelet morphology
- 31. 3.c Laboratory A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a 3rd step→Platelet morphology Robier, C., 2020. Platelet morphology. Journal of Laboratory Medicine, 44(5), pp.231-239. (A–E) Megakaryocytes; (F–H) micromegakaryocytes; (I) megakaryoblasts. May-Hegglin anomaly (A large platelet and three mature neutrophils with large cytoplasmic May-Hegglin inclusions, which resemble Döhle-bodies)
- 32. 3.d Laboratory A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Indications for bone marrow biopsy • Red flag symptoms (fever, night sweats, weight loss, bony pains, and fatigue) • Involvement of other blood cell lines (anemia or neutropenia) • Blasts in peripheral blood smear • The cause of thrombocytopenia is unclear.
- 33. Plat< 150,000 ina child No plat clumps No anemia or pancytopenia Critical ill Macrothrombocytes Not critical ill No congenital anomalies or drugs See morphology + size No Macrothrombocytes PT, PTT, INR ↑ spleen → Malaria, portal HTN, Gaucher disease ↑ liver & lymphadenopathy → lymphoma, leukemia, MDS Prolonged Normal Acute febrile illness – chronic ill Spleen size & liver size & lymph nodes Acute fever → sepsis, EBV, CMV, HIV, parvovirus Chronic ill → HIV, HUS/TTP, autoimmune or connective tissue disease
- 34. 4. Genetic A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Indications: Platelet dysfunction/defect Abnormal bleeding Unexplained thrombocytopenia Easy bruising/spontaneous ecchymoses Positive family history of bleeding disorders or platelet function disorders Gene Sequencing www.cincinnatichildrens.org/genetics
- 35. 4. Genetic A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Gene Sequencing www.cincinnatichildrens.org/genetics
- 36. 4. Genetic A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Genetic Conditions Commonly Associated with Plat Disorders www.cincinnatichildrens.org/genetics
- 37. 4. Genetic A pp r o a c h t o p e d i a t r i c p a t i e n t s w i t h t h r o m b o c y t o p e n i a Genetic Conditions Commonly Associated with Plat Disorders www.cincinnatichildrens.org/genetics
- 38. What is themost common cause of Thrombocytopenia? Approach to pediatric patients with thrombocytopenia
- 39. When can yourequest pediatric hematologist consultation? Approach to pediatric patients with thrombocytopenia
- 40. Algorithm for pediatricthrombocytopenia diagnosis Approach to pediatric patients with thrombocytopenia
- 41. Approach to pediatric patientswith thrombocytopenia Indications for platlet transfusion 1) Platelet transfusion threshold in bleeding patients 2) Prophylactic Transfusion threshold 3) Platelet Transfusion in Specific Settings
- 42. Approach to pediatric patientswith thrombocytopenia Indications for platlet transfusion 1- Platelet transfusion threshold in bleeding patients • < 50,000 cells/microliter in sever bleeding including DIC. • < 30,000 cells/microliter when bleeding not life-threatening or not severe. • < 100,000 cells/microliter in bleeding in multiple trauma patients or with intracranial hemorrhage. Atif. Khan; Faiz Answer. Platelet transfusion 2023
- 43. Approach to pediatric patientswith thrombocytopenia Indications for platlet transfusion 2- Prophylactic Transfusion threshold 1) To prevent spontaneous bleeding (< 10,000 cells/microliter/some recommend < 5000 cells/microliter). 2) Before neurosurgery or ocular surgery (< 100,000 cells/microliter). 3) Before major surgery (< 50,000 cells/microliter). 4) In DIC (< 50,000 cells/microliter). 5) Before central line insertion (< 20,000 cells/microliter). 6) Before epidural anesthesia (< 80,000 cells/microliter). 7) Before bronchoalveolar lavage (BAL) (20,000 to 30,00 cells/microliter). 8) Before endoscopic procedures: • <50,000 cells/microliter for therapeutic procedures. • <20,000 cells/microliter for low-risk diagnostic procedures. 9) Vaginal delivery platelet transfusion is considered at <30,000 cells/microliter, and when traumatic delivery, then <50,000 cells/microliter. 10) Before lumbar puncture - less than 20,000 cells/microliter in patients with hematologic malignancies and 40,000 to 50,000 cells/microliter in patients without hematologic malignancies. 11) Platelet transfusion is not routinely indicated prior to bone marrow biopsy, peripheral or central catheter insertion, traction removal of tunneled central venous catheters, and cataract removal. Atif. Khan; Faiz Answer. Platelet transfusion 2023
- 44. Approach to pediatric patientswith thrombocytopenia Indications for platlet transfusion 3- Platelet Transfusion in Specific Settings 1) ITP - transfusion is avoided unless severe bleeding is present. 2) Malignancy and chemotherapy - In most cancers, platelet transfusion thresholds are as indicated above, except in acute promyelocytic leukemia, in which there is increased bleeding risk. Hence transfusion is indicated at counts <30,000 cells/microliter. 3) Cardiac surgery - Patients undergoing cardiac surgery get exposed to a blood-pumping circuit, which activates platelets that get destroyed once back in circulation; hence even at normal counts, platelet transfusion is indicated during cardiac surgery. 4) Inherited and acquired platelet disorders like Glanzmann thrombasthenia, Bernard-Soulier syndrome, and other congenital platelet defects, acquired platelet disorders like4.patients with uremia or drug-induced platelet dysfunction. In these situations, platelet transfusion is indicated only when bleeding Is present. 5) For patients in the following situations, transfus platelets when the platelet count is: • <30,000 cells/microliter in neonates without any bleeding or symptom and failure to produce platelet. • <50,000 cells/microliter in an infant with active bleeding or undergoing an invasive procedure. For the same situation in a premature infant, we transfuse at less than 100,000 cells/microliter. • Patient undergoing extracorporeal membrane oxygenation (ECMO) and platelets <100,000 cells/microliter. Atif. Khan; Faiz Answer. Platelet transfusion 2023
- 45. Approach to pediatric patientswith thrombocytopenia Contraindications for platlet transfusion 1) The only agreed upon contraindication to platelet transfusion is TTP due to the increased risk of thrombosis, although studies on outcomes and mortality have shown mixed results. Platelet transfusion is reserved for life-threatening bleeding only. 2) Heparin-induced thrombocytopenia (HIT) is another condition where platelet transfusion may increase the risk of thrombosis, but recent studies have shown no risk association. In HIT, transfusion is reserved only for pre-procedure or surgery, and in severe bleeding, prophylactic transfusion, however, is not indicated.
- 46. Approach to pediatric patientswith thrombocytopenia Preparations for platlet transfusion 1) The shelf life of platelet concentrates (PC) is 5 days, within which it must be used. 2) The normal dose of platelet transfused is calculated as 10 to 15 ml/kg of the patient. 3) Consent from the patient must be obtained before sending a request to the blood bank 4) A pretransfusion sample will be checked for ABO and Rh blood grouping. 5) A group-specific PC is recommended, although out of the group can also be issued. 6) Serologic crossmatch is not required except in rare cases where PC has high RBC content. 7) Special requirements such as leukoreduction to reduce HLA alloimmunization or to minimize CMV transmission and irradiation to prevent transfusion-associated graft vs. host disease (TA-GvHD) might be needed in specific patient groups (eg, hematological malignancies, BMT). 8) Staff should do the final checks for details such as patient ID, unit no, blood group, and abnormal appearance or clumps suggestive of infection in the PC bag before using the unit.
- 47. Approach to pediatric patientswith thrombocytopenia Family Education: 1) Provide written information to document diagnosis. 2) Restrict activities to minimize the risk of head injury: • Avoid sports (eg football, boxing) • Limit contact sports that have a risk for traumatic injury (eg, horse-riding, riding scooter, skateboard, or bike, climbing) 3) Avoid anti-platelet (aspirin), NSAIDs (ibuprofen), anti-coagulants. 4) Avoid intramuscular injections. 5) Monitor for significant bleeding symptoms and go immediately to ER if they occur. 6) Monitor for signs of ICH and go immediately to ER if head injury or severe headache
- 48. Immune thrombocytopenia Approach topediatric patients with thrombocytopenia
- 49. Immune thrombocytopenia Past(idiopathic thrombocytopenic purpura) Diagnosis: if the platelet counts are repeatedly below 100 × 109/µL The detection of isolated thrombocytopenia in the presence of otherwise normal leukocyte and erythrocyte parameters is usually sufficient for an initial diagnosis A blood smear must always be examined. Gender: o In pediatric boys are more often affected than girls o In middle age, women are more likely to develop ITP than men. After age 60 years, men predominate again. Pathophysiology Approach to pediatric patients with thrombocytopenia
- 50. Immune thrombocytopenia ClinicalPresentation o The central clinical symptom of ITP is the increased bleeding tendency. o Petechiae and mucosal hemorrhages are typical. o Many ITP patients also complain of exhaustion and fatigue, including depressive disorders Definition of bleeding manifestations Grading the Severity of Bleeding Approach to pediatric patients with thrombocytopenia
- 51. Immune thrombocytopenia Prognosisand risk indicators Phases of the disease and treatment goals Indications for bone marrow biopsy Approach to pediatric patients with thrombocytopenia
- 52. Immune thrombocytopenia Pre-treatmentconsidered factors First line therapy o Early response: plat count ≥ 30 x 109/L and at least doubling baseline at 1 week o Initial response: plat count ≥ 30 x 109/L and at least doubling baseline at 1 month o Durable response: plat count ≥ 30 x 109/L and at least doubling baseline at 6 months o Remission: plat count > 100 x 109/L at 12 months Definition of response Approach to pediatric patients with thrombocytopenia
- 53. Immune thrombocytopenia Pre-treatmentconsidered factors First line therapy o Early response: plat count ≥ 30 x 109/L and at least doubling baseline at 1 week o Initial response: plat count ≥ 30 x 109/L and at least doubling baseline at 1 month o Durable response: plat count ≥ 30 x 109/L and at least doubling baseline at 6 months o Remission: plat count > 100 x 109/L at 12 months Definition of response Approach to pediatric patients with thrombocytopenia
- 54. Immune thrombocytopenia Diagnosticworkup for patients with persistent or chronic ITP Approach to pediatric patients with thrombocytopenia
- 55. Second-Line Treatment with TRAs Approachto pediatric patients with thrombocytopenia
- 57. Thrombocytosis Tips & Tricksin CBC reading
- 58. Thrombocytosis Tips & Tricksin CBC reading Thrombocytosis (> 450,000) Mild > 500,000 Moderate > 700,000 Severe > 900,000 Extreme > 1000,000 Etiology: Primary (caused by a process that is intrinsic to megakaryocyte) Reactive (caused by a process that is extrinsic to megakaryocyte) Thrombocytosis in children is almost always a reactive response, most often infectious or inflammatory (interleukin-6 & c-reactive protein). 1/3 of the circulating platelets are normally sequestrated within the spleen. Stress like exercise or surgery are commonly cause thrombocytosis. Down syndrome, ↑ plat is very common by the 2nd month of life for 1st year.
- 59. Thrombocytosis Tips & Tricksin CBC reading Primary thrombocytosis is very rare: o Familial: mutation in thrombopoietin gene (TPO) or MPL o Acquired: Essential thrombocythemia, polycythemia vera, primary myelofibrosis, chronic myeloid leukemia, MDS Essential thrombocytosis is also very rare (diagnosis of exclusion). Hematological disorders for reactive thrombocytosis: o Iron deficiency anemia (at diagnosis or during therapy) o Megaloblastic anemia o Hemolysis o Sickle hemoglobinopathies (functional asplenia→ Howell-jolly bodies) Initial labs: cbc, retic, peripheral smear, ESR, hepatic functions, urine analysis, cxr.
- 60. Always put yourselfin the others shoes. If you feel that it hurts you, it probably hurts the person too.
- 61. If you don’tuse it, you lose it Tips & Tricks in CBC reading
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