PATIENTMANAGEMENTPATIENT MANAGEMENT PANCYTOPENIA · Abd :...
Transcript of PATIENTMANAGEMENTPATIENT MANAGEMENT PANCYTOPENIA · Abd :...
PATIENT MANAGEMENTPATIENT MANAGEMENT
PANCYTOPENIA
25 Feb 2015Sirirat Assawalerknun
HISTORYHISTORY เด็กหญิงไทยอายุ 11 ป no underlying disease
ิ ํ ศ ีสภูมิลําเนา จ.ศรีสะเกษ
อาการสาํคญั: สงตัวจากรพ.รัฐบาลมารักษาตอดวยเรื่องซีดและ ญ ฐ
เกร็ดเลือดต่ํา
HISTORYHISTORY ประวตัปิจจบุนั
3 ื ี ื ี ศี ี ้ํ ื ึ้3 เดือนกอน มีอาการหนามืด เวียนศีรษะ มีจุดจําเลือดขึน
ตามตัว ไมมีถายดําหรืออาเจียนเปนเลือด ปฎิเสธประวัติไข ไอฎ
น้ํามูก หรือการเจ็บปวยกอนหนานี้
ไป admit รพ.ใกลบาน
HISTORYHISTORYInvestigation:
1 CBC : Hct 19% WBC 4 100 /mm3 (N14% L81%)1. CBC : Hct 19%, WBC 4,100 /mm3 (N14%,L81%),
ANC 574 /mm3 , Platelet 10,000 /mm3
2. DAT : neg, IAT : neg
3 LDH 1673. LDH : 167
4. BMA : dilute marrow, not seen blast cell
HISTORYHISTORYManagement:
LPB 6 unit LPB 6 unit
Platelet concentrate 40 unit
F/U CBC (กอน D/C)
CBC H t 23 5% WBC 2 700 / 3 ( N9% L87%)CBC : Hct 23.5%, WBC 2,700 /mm3 ( N9%,L87%),
ANC 243 /mm3 ,Platelet 68,000 /mm3
Imp : Pancytopenia
Refer to siriraj hospital
HISTORYHISTORY ประวัติอดีต : ปฎิเสธการเจ็บปวยรุนแรงกอนหนานี้
ประวัติคลอด : ครบกําหนด ไมมีภาวะแทรกซอนหลังคลอด ประวตคลอด : ครบกาหนด ไมมภาวะแทรกซอนหลงคลอด
ประวัติครอบครัว : บิดาเปนพาหะธาลัสซีเมีย
ประวัติวัคซีน : ครบตามเกณฑ
ประวัติยา : ไมไดใชยาใดเปนประจํา
PHYSICAL EXAMINATION (siriraj)PHYSICAL EXAMINATION (siriraj)
V/S : BT 36.5 o C, RR 22 /min, HR 102 /min, BP 108/73 mmHg ,
BW 34.5 Kg (P50), Lt 149 cm (P50-75) g ( ) ( )
GA : alert, markly pale, no jaundice, generalized petechiae,
no ecchymosis, no lymphadenopathy, no rash, no oral ulcer
RS : clear, equal both
CVS : normal S1S2, SEM gr II/VI at LUPSB
Abd : soft no palpable mass no hepatosplenomegalyAbd : soft, no palpable mass, no hepatosplenomegaly
NS : E4V5M6, pupil 3 mm BRTL, motor power Gr V/V all
PROBLEM LISTPROBLEM LIST
Pancytopenia
SEM gr II/VI at LUPSB SEM gr II/VI at LUPSB
INVESTIGATIONINVESTIGATION
CBC
Hb 9.9 g/dl
Hct 28.9%
MCV 79 fl
WBC 2,930 /mm3
N 8.3 %
L 88.7%
ANC 243 /mm3ANC 243 /mm
Plt 22,000 /mm3
Reti 0 2%Reti 0.2%
PANCYTOPENIA
PANCYTOPENIAPANCYTOPENIA
Definition
Hemoglobin (Hb) < 10 g/dl
Absolute neutrophil count < 1.5 x109/l
Pl t l t t < 100 109/l Platelet count < 100 x109/l
Pancytopenia in children, Pak J Med Sci 2013 Vol. 29 No. 5
Manual of Pediatric Hematology and Oncology Fifth Edition
Journal, Indian Academy of Clinical Medicine, 2012; Vol13 No. 4
ETIOLOGYETIOLOGY
Indian Journal of Pathology and Microbiology - 54(1)76,January-March 2011
Aplastic anemiaAplastic anemia
Acute leukemia
Bone marrow
Megaloblastic anemiaMegaloblastic anemia
Hypersegmentalneutrophil
Peripheral Blood smearneutrophil
Gaucher's Disease
Storage diseaseStorage disease
Niemann–Pick disease
Granuloma
PANCYTOPENIAPANCYTOPENIA
Anemia : pallor, weakness, loss of appetite
Leukopenia : increased susceptibility to infections
Thrombocytopenia : petechiae, easy bruising,
severe nosebleeds and bleeding into GI andsevere nosebleeds and bleeding into GI and
renal tracts
SYMPTOMS AND SIGNSSYMPTOMS AND SIGNS
Indian Journal of Pathology and Microbiology - 54(1)76,January-March 2011
INVESTIGATION OF PANCYTOPENIAINVESTIGATION OF PANCYTOPENIA
Laboratory CBC CBC
Reticulocyte count
Peripheral blood smear
Bone marrow exam (aspiration/biopsy)
INVESTIGATION OF PANCYTOPENIAINVESTIGATION OF PANCYTOPENIA
Further LaboratoryA t i ANA DNA tit DAT Autoimmune : ANA , DNA titer, DAT
Infection : Viral serology eg. HIV, EBV, parvovirus, hepatitis profile,
PCR for virus
Nutritional def : Serum vitamin B12, serum folate levels
X-Rays: Bone, chest etc / ultrasound abdomen
Other biopsies : Lymph node Other biopsies : Lymph node
Fanconi anemia : Chromosome breakage
IN THIS PATIENTIN THIS PATIENT
BM biopsy
♧ d h ll l i (20 25% f l )♧ moderate hypocellularity (20-25% of total marrow)
♧ marked decrease in megakaryocytesg y y
♧ predominance of erythroid precursors with maturation
♧ decrease in myeloid precursors
♧ ti f t d l♧ negative for tumor and granuloma
DIAGNOSISDIAGNOSIS
Severe aplastic anemiaCriteriaAt least 2 of following peripheral blood findings: Reticulocytes <1%, corrected for hematocrity %, Absolute neutrophil count < 0.5×109/L Platelets < 20×109/L Platelets < 20×10 /LAND
BM biopsy with < 25% normal cellularity OR BM biopsy with < 25% normal cellularity OR BM biopsy with <50% normal cellularity in which less
th 30% f ll h t i tithan 30% of cells are hematopoietic
MANAGEMENTMANAGEMENTOF PANCYTOPENIAOF PANCYTOPENIA
MANAGEMENTMANAGEMENT
Supportive treatment Specific treamentp
SUPPORTIVE RXSUPPORTIVE RX
Anemia : blood transfusion Neutropenia :p
♤ Prophylaxis ATB♤ Treament of Febrile neutropenia♤ Treament of Febrile neutropenia♤ Granulocyte concentrates
Thrombocytopenia/Bleeding : ♤ Platelet concentrates
Hygiene
TransfusionTransfusion
Transfusions Only when indicated Side effects:
- Iron overload - HLA alloimmunization, - Infection transmission (eg : HIV,HCV,HBV,CMV)
TransfusionTransfusion
Irradiation ….. Leukocyte reduced blood (LRB) …..y ( )
TransfusionTransfusion
Irradiation of blood products
♧ To prevent transfusion associated GvHD (TA GvHD)♧ To prevent transfusion-associated GvHD (TA-GvHD)
♧ To reduce sensitization to HLA and non-HLA antigens
from multiple transfusions in patients who are candidates
f t l t d f ATG t t tfor transplant and for ATG treatment
Irradiation of blood productsIrradiation of blood products
I f t 6 th f Infants < 6 months of age All pediatric malignancies
I i l h f i Intrauterine or neonatal exchange transfusions Recipients of components from blood relatives and all designated
donorsdonors Recipients of crossmatched or HLA matched platelets Myelosuppressive therapy (chemotherapy or irradiation) Myelosuppressive therapy (chemotherapy or irradiation) Patients undergoing, or candidates for, marrow or peripheral blood
progenitor cell transplant H Congenital immunodeficiencyprogenitor cell transplant H. Congenital immunodeficiency syndromes, include suspected DiGeorge Syndrome
PEDIATRIC TRANSFUSION GUIDELINES (Approved by Medical Staff Executive Committee on 12/11/2006)
Leukocyte reduced bloodLeukocyte reduced blood
Leukocyte reduced blood (LRB) - leukocyte-poor packed red cell, LP-PRC
: < 5 x 108 WBC/bag- leukocyte-depleted packed red cell, LD-PRC
: < 5 x 106 WBC/baga) pre-storage filtration ) p gb) bedside filtration
Leukodepleted blood componentsLeukodepleted blood components
Recommended♣ Prevent of FNHTRs (Febrile non haemolytic transfusion reactions)♣ Reducing graft rejection after haemopoietic cell transplantation.♣ Prevention of transmission of CMV infections.♣ Fetal/Neonatal transfusions.
Possible♠ Prevention of refractoriness to platelet transfusion♠ Kidney transplants.♠ Immunomodulation.
Recommendations for the transfusion of red blood cells 2009; 7: 49-64
ANEMIAANEMIA
Recommendations for the transfusion of red blood cells 2009; 7: 49-64
Blood TransfusionBlood Transfusion
A safe haemoglobin level ≥ 8 g/dl,
Based on symptoms and co-morbidities; quality of life.
Patients with cardiac pulmonary vascular co morbidities Patients with cardiac, pulmonary, vascular co-morbidities
may require a higher transfusion.
Regular blood transfusion is associated with risk of
alloimmunization and iron overload.
Blood TransfusionBlood Transfusion
Dose ….
Premedication ….
Blood TransfusionBlood Transfusion
Dosage :
10 cc/kg will increase hemoglobin 2.5-3.0 g/dl.10 cc/kg will increase hemoglobin 2.5 3.0 g/dl.
Transfusion time:
In uncomplicated patients infuse over 2-3 hours.
HCC syndrome (hypertension convulsion cerebral y ( yp
hemorrhage syndrome)
Blood TransfusionBlood Transfusion
ผูปวยที่มีปญหาการทํางานของหัวใจ และระดับ pretransfusion Hb < 5 g/dL
1. ใหปริมาตร PRC ที่ใหเทากับขนาด 2 เทาของระดับ Hb แตไมเกิน 5 mL/kg
2. อัตราการให < 2 mL/kg /ชั่วโมง โดยใหปริมาณนอย แตบอยครั้ง (ทุก 24–48 ชม.)
3. พิจารณาใหยาขับปสสาวะกอนให PRC
ผที่มีปร วัติ febrile non hemol tic transf sion reaction (FNHTR) ให ผูทมประวต febrile non-hemolytic transfusion reaction (FNHTR) ให
Chlorpheniramine และ Paracetamol รับประทานกอนใหเลือด 1/2-1 ชม.
ถามีอาการ FNHTR ขณะใหเลอืดใหหยุดการใหเลอืดทันที และวัด vital signs
Platelet TransfusionPatients Recommendation
Platelet Transfusion
Leukemia/Lymphoma
<10x109/L for clinically stable patients receiving CMT<40x109/L with signs of bleeding, high fever, critically ill
hyperleucocytosis rapid fall in platelet count APL hyperleucocytosis, rapid fall in platelet count, APL, coagulation abnormality (*)
Post stem cellt l t ti
<10x109/L hi h l l i f ti t b (*)transplantation higher level : require for patient as above (*)higher level : Patients undergoing invasive procedures
Solid tumors <10x109/L higher level : require for patient as above (*)higher level : Patients undergoing invasive procedureshigher level : for patients with bladder tumors or necrotic tumors
Chronicthromboctyopenia
Stable patients with chronic, stable, severe thrombocytopeniadue to alloimmunization should be observedPlatelet transfusions with clinically significant bleeding only.
Guideline for Platelet Transfusion Thresholds for Pediatric Hematology/Oncology Patients 2011
Platelet TransfusionPatients Recommendation
Platelet Transfusion
Lumbarpuncture
<20x109/L<50x109/L : recommend for diagnostic LP for newly diagnosed patients with leukemia to minimize risk of traumatic LPpatients with leukemia to minimize risk of traumatic LP.higher level : require for patient as above (*)
Majori i
<40-50 x109/L hi h l l i f ti t b (*)invasive
procedurehigher level : require for patient as above (*)
Guideline for Platelet Transfusion Thresholds for Pediatric Hematology/Oncology Patients 2011
NeutropeniaNeutropenia
Prophylaxis ATB Treament of Febrile neutropenia Treament of Febrile neutropenia Granulocyte concentrates
ATB ProphylaxisATB Prophylaxis
Prophylactic antibiotic and antifungal drugs should
be given to patients with ANC <0 5X109/lbe given to patients with ANC <0.5X109/l.
ATB ProphylaxisATB Prophylaxis
ATB ProphylaxisATB Prophylaxis
Prophylactic antibiotics prevent Gram-negative sepsis, ♧ combination of two non-absorbable antibiotics:
neomycin and colistin♧ quinolone : ciprofloxacinq p
Concern about emergence of ♧ quinolone resistant bacteria♧ quinolone resistant bacteria, ♧ increase in Gram-positive infections♧ increase risk of Clostridium difficile♧ increase risk of Clostridium difficile
Ciprofloxacin cannot be used to treat febrile neutropenia if it is used prophylactically
Antifungal ProphylaxisAntifungal Prophylaxis
Aplastic anaemia are high risk of fungal infection, including Aspergillus.
Fluconazole: no cover against Aspergillus species. Drugs of choice are itraconazole and posaconazole.g p No data to justify use of voriconazole for prophylaxis
Febrile neutropeniaFebrile neutropenia
Febrile neutropeniaFebrile neutropenia
Fever with neutropenia is an indication for hospitalization.
Diagnosis : physical exam, H/C and cultures from other sites, CXRg p y , ,
Start treatment without waiting for culture results.
Persisting fever or previous or suspected fungal infection Persisting fever or previous or suspected fungal infection
- Diagnostic procedures for invasive fungal infections :
galactomannan and CT chest should be performed.
Systemic antifungal therapy concern early in therapy of fever in
neutropenic AA patients.
Febrile neutropeniaFebrile neutropenia
Antibiotics :
♤ synergistic combination such as aminoglycoside and♤ synergistic combination such as aminoglycoside and
b-lactam penicillin
♤ depending on local hospital microbiological sensitivity/
resistance patternsresistance patterns.
G-CSFG CSF
C id d f i i f i h Considered for severe systemic infections that are not responding to intravenous ATB and anti-fungal drugs
Short course of subcutaneous G-CSF : dose of 5 mcg/kg per day
Discontinued after 1 week if there is no increase in neutrophil counts
Granulocyte transfusionsGranulocyte transfusions
Considere in life-threatening neutropenic sepsis. Adverse events
# febrile reactions, # HLA alloimmunization # transfusion-related acute lung injury (TRALI)
Supportive careSupportive careS i l N t i di t h Special Neutropenic diet such as: (1) washing hands before preparing food, (2) storing raw meat fish chicken in a way that avoids(2) storing raw meat, fish, chicken in a way that avoids
contamination of other food, (3) washing in hot water dishes and cutlery that contact raw meat
before placing them in contact with other food,(4) using fresh food without any mold, (5) consumption of Pasteurized juices and dairy products, (6) avoiding uncooked meats, seafood, eggs and unwashed fruits
and vegetablesand vegetables (7) avoiding raw nuts and dried fruits due to the risk of fungal spores.
Supportive careSupportive care
Hand washing and rubbing with alcohol-based disinfection
must be used before and after handling the patient by
staff and by visitors.
HygieneHygiene
oral hygiene :- soft toothbrushes to avoid bleeding, lesions of mucosa- Regular mouth care: antiseptic mouthwash
ActivitiesActivities
Early mobilization to avoid bed sores and guarantee
good pulmonary functiongood pulmonary function.
Physical exercises are recommended if the patient is
well enough.
Passive mobilization and breathing may be helpful for Passive mobilization and breathing may be helpful for
hospitalized patients who are not able to perform such
physical activities.
Psychological supportPsychological support
support of patients and families
age appropriate explanation of disease treatment and age-appropriate explanation of disease, treatment, and
prognosis are important at diagnosis and during course
of disease
improve therapy adherence and disease outcomes improve therapy adherence and disease outcomes.
SPECIFIC RXSPECIFIC RX
Depend on underlying diseases Severe aplastic anemiap
- HLA-identical sibling BMT- Immunosuppressive therapy: pp py
antithymocyte globulin (ATG) and ciclosporin
การพยาบาลผปวยที่มีภาวการพยาบาลผูปวยทมภาวะ THROMBOCYTOPENIATHROMBOCYTOPENIA
THANK YOUTHANK YOU