Hematologic and

Published on Nov 18, 2015

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PRESENTATION OUTLINE

Sickle Cell

hemoglobinpathy
Sickle Cell:
Caused by a gene in which there is a structurally abnormal portion of the hemoglobin chain.

Risk Factor: Being Heterozygous for hemoglobin S or being of African American Descent.

-clinical screening: Sickledex: run over about 3 minutes from a finger stick.

Hemoglobin S is sensitive to changes in the oxygen content in the red blood cell.

Insufficient oxygen causes the cells to assume a sickle shape, and the cells become rigid and clumped together , obstructing capillary blood flow.

https://www.khanacademy.org/science/health-and-medicine/hematologic-system-...
Photo by scooterdmu

Sickle Cell

Crisis
Precipitating factors:
fever, dehydration, emotional and or physical stress, conditions that increase the need for oxygen

" vaso occlusive crisis, splenic sequestration, hyerhemolytic crisis, aplastic crisis"


Photo by Zoriah

crisis

  • Vaso-Occlusive: clumping, ischemia, infarction
  • Splenic Sequestration: Profound Anemia, Hypovolemia, and SHOCK
  • Hyperhemolytic: An accelerated Rate of red blood cell destruction (anemia, jaundice, and reticulocytosis)
  • Aplastic -diminished production or increased destruction
Photo by Janesdead

FUnctional

Asplenia
Sickle Cell kids need to have their vaccines all up to date, because of their increased risk of functional asplenia.

http://emedicine.medscape.com/article/885226-overview

Iron deficiency

Anemia
comes from:
blood loss, increased metabolic demands (pregnancy!), GI malabsorption issues (Cystic Fibrosis!), poor diet

clinical presentation: pallor, weakness and fatigue, low hemoglobin, and hematocrit levels

RBC's that are microcytic and hypochromic

black stool stools.
Photo by AMagill

iRON dEFICIENCY aNEMIA

BREAST fED BABIES
Photo by jcarter

iron rich

Foods! 
breads, whole grains, dark green leafy vegetables, dried fruits, egg yolks, kidney beans, legumes, liver, meats, molasses, nuts, potatoes, prune juice, raisins, seeds, shellfish, Tofu

iron fortified formula and cereal,

iron supplement:
give between meals for maximum absorption
give with a multi vit or fruit juice because vit C increases absorption, Do not give with milk or antacids because they decrease absorption.

can cause constipation.

Liquid Iron should be taken via STRAW it will STAIN your teeth.

Aplastic

Anemia, what's going on with the bone Marrow
cause:
exposure to myelotoxic agents
viruses
infection
auto immune disorders
allergic states.

dx: bone marrow aspiration: will show conversion of red bone marrow to fatty bone marrow.

treatment: immunosuppressive therapy, and bone marrow transplant (treatment of choice)

assessment: Pancytopenia: deficiency of erthrocytes, leukocytes, and thrombocytes

Hemophilia, bleeding disorder

https://www.khanacademy.org/search?search_again=1&page_search_query=Hemophi...

http://www.mayoclinic.org/diseases-conditions/hemophilia/basics/definition/...

https://www.khanacademy.org/test-prep/mcat/organ-systems/hematologic-system...

There are many different coagulation components: which one is deficient or absent is essential in treating hemophila.

treatment: replace clotting factor that is missing, additional medication agents such as: pain meds, and corticosteroids

(FACTOR VIII concentrates, DDAVP (synthetic vasopressin),
epitaxis ( chronic is ominous- and can be potentiated by steroids)
monitor for blood in the urine,
Photo by bayat

Type of Hemophilia

why Christmas Disease?
Hemophilia A
“Classic hemophilia”
Deficiency of factor VIII
Hemophilia B
Also known as “Christmas disease”
Caused by deficiency of factor IX
Von Willebrand disease
Deficiency of von Willebrand factor and factor VIII

Christmas Disease:
http://www.healthline.com/health/hemophilia-b#Overview1
The disease has nothing to do with the Christmas holiday; it was named for Stephen Christmas, who was the first person diagnosed with the condition in 1952. Christmas disease is the second most common form of hemophilia. Approximately one in 3,300 people in the United States have Christmas disease, according to the National Hemophilia Foundation. (NHF)
Photo by scottfidd

Von Willebrand

defect in a protein "von willebrand Factor"
characterized by bleeding mucous membranes:

epistaxis, gum bleeding, easy bruising, excessive menstrual bleeding

treatment similar to treatment for hemophilia- especially use of clotting factors.

types of Thalassemia

  • Thalassemia Minor: Asymptomatic silent carrier Case
  • Thalassemia Trait: Produces mild microcytic anemia
  • Thalassemia INtermedia: Manifested as splenomegaly and moderate to severe anemia
  • Thalassemia Major: Results in severe Requiring transfusion support to sustain Life (cooley's Anemia)
http://www.cdc.gov/ncbddd/thalassemia/

Anemia results from defective synthesis of Hgb, structural impairment of RBCs, and shortened life of RBCs

Thalasemia

autosomal Recessive
* https://www.nlm.nih.gov/medlineplus/ency/article/003301.htm
Frontal Bossing

physical symptoms:
Maxillary Prominence
Wide-set- eyes with a flattened nose
Greenish Yellow skin tone
Hepatosplenomegaly
Severe Anemia
Microcytic, hypochromic red blood cell

Normal postnatal hemoglobin includes two -thalassemia and two -thalassemia chains
Anemia results from defective synthesis of Hgb, structural impairment of RBCs, and shortened life of RBCs
Overabundance of erythrocytes is found in the bone marrow
Photo by kainr

lab value

how low is too low?
Diagnostic evaluation
Sometimes defined as Hgb
Photo by kylesteed

Suspicion

Upon assessment and taking health history these things would make you suspect a blood disorder:

Complete blood cell count History and physical examination
Comments by the parent regarding child’s lack of energy
Food diary of poor iron sources
Frequent infections
Bleeding
Photo by paulbence

eFFECTS OF aNEMIA ON cIRCULATORY sYSTEM

  • Hemodilution Decreased peripheral
  • resistance
  • Increased cardiac circulation and turbulence
  • Such increases may produce a murmur Cardiac failure may ensue
  • Cyanosis
  • Growth retardation

Idiopathic

Thrombocytopenic Purpura
Also known as “idiopathic thrombocytopenic purpura"
An acquired hemorrhagic disorder characterized by
Thrombocytopenia: excessive destruction of platelets
Purpura: discoloration caused by petechiae beneath the skin
Normal bone marrow with increased number of immature platelets or eosinophils



http://www.webmd.com/a-to-z-guides/thrombocytopenia-symptoms-causes-treatme...

Therapeutic management
Primarily supportive
IV immune globulin
Anti-D antibody

DIC

Disseminated Intravascular Coagulation
https://www.khanacademy.org/science/health-and-medicine/circulatory-system-...

Sepsis and DIC

Disorder of coagulation that occurs as complication of numerous pathologic processes
Hypoxia
Acidosis
Shock
Endothelial damage

First stage of the coagulation process is abnormally stimulated
Excessive amounts of thrombin are generated
Fibrinogen is converted rapidly to fibrin, with aggregation and destruction of platelets
Theraputic Management of DIC: Early recognition
Control of the underlying or initiating cause
Platelet or fresh frozen plasma transfusion
Exchange transfusion in the newborn
IV heparin
Photo by jlwo

epitaxis

nosebleed
Isolated and transient epistaxis is common in childhood
Recurrent or severe episodes may indicate underlying disease
Vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiency diseases (von Willebrand disease and hemophilia)


Remain calm; keep child calm
Bleeding usually stops within 10 minutes after nasal pressure
Have child sit up and lean forward
Apply pressure to the soft lower part of the nose
Evaluate further if bleeding continues
Photo by schoschie

CANCER

Neoplastic Disorders
Leading cause of death from disease in children past infancy
Almost half of all childhood cancers involve blood or blood-forming organs
Leukemia and lymphomas

Leukemias

Most common form of childhood cancer
Three or four cases per 100,000 white children More frequent in boys >1 year old
Peak onset between 2 and 5 years of age
Survivability: currently 60% - used to be a death sentence

someone gets a blood cancer every 3 minutes
every 9 minutes someone dies from a blood cancer.


https://www.lls.org/http%3A/llsorg.prod.acquia-sites.com/facts-and-statisti...


https://www.khanacademy.org/science/health-and-medicine/hematologic-system-...
Photo by MattBritt00

Morphology

of Leukemia
Acute lymphoid leukemia (ALL)
Acute nonlymphoid (myelogenous) leukemia (ANLL or AML)
Stem cell or blast cell leukemia
Pathophysiology of Leukemia:
Leukemia is an unrestricted proliferation of immature WBCs in the blood-forming tissues of the body
Liver and spleen are the most severely affected organs
Leukemia demonstrates the same neoplastic properties as solid tumors

Although leukemia is an overproduction of WBCs, often acute form causes low leukocyte count
Cellular destruction takes place by infiltration and subsequent competition for metabolic elements
Photo by franz88

wait? Leukemia

is a form of Anemia???
Anemia from decreased RBCs--> blood product manufacturing has been compromised.

Infection from neutropenia
Bleeding tendencies from decreased platelet production
Marked infiltration, enlargement, and fibrosis of spleen, liver, and lymph glands


Care Management:
Prepare child and family for procedures
Relieve pain
Prevent complication of myelosuppression
Infection
Hemorrhage
Anemia
Use precautions with chemotherapeutic agents
Photo by Watchcaddy

Bone Marrow

Biopsy
Diagnostics for Leukemia:
Based on history, physical manifestations
Peripheral blood smear
Immature leukocytes
Frequently low blood cell counts
Lumbar puncture to evaluate central nervous system (CNS) involvement
Bone marrow aspiration or biopsy

Therapy: Four phases of therapy
Induction therapy: 4-6 weeks
Central nervous system prophylactic therapy: intrathecal chemotherapy
Intensification (consolidation) therapy: to eradicate residual leukemic cells and prevent resistant leukemic clones
Maintenance therapy: to preserve remission

HSCT

Hematopoietic Stem Cell Transplant
Donors: may be relatives or nonrelatives
Antigen matched or mismatched
Peripheral stem cells: may be used
Umbilical cord blood
Graft-versus-host disease
Severe organ damage

prognosis qualifiers
Factors for determining prognosis
Initial WBC count
Age at time of diagnosis
Type of cell involved
Gender
Karyotype analysis
Photo by Rene Mensen

life goes on

Care Management role of the nurse
Manage problems of drug toxicity
Nausea/vomiting
Anorexia
Mucosal ulceration
Neuropathy
Hemorrhagic cystitis
Alopecia
Mood changes
Moon facies


what can you do to help manage these side effects of drug therapy?
Photo by Kevin_Morris

Lymphoma

third most common group of Malignancy
Third most common group of malignancies in children
Hodgkin lymphoma
More prevalent among children 15-19 years of age
Non-Hodgkin lymphoma
More prevalent among children

Neoplastic disease originating in lymph system
Primarily involves lymph nodes
Often metastasizes to spleen, liver, bone marrow, lungs, and other tissues
Photo by Jeremy Brooks

Four Types of Hodgkin Lymphoma

  • 1) Lymphocytic predominance 2) Nodular sclerosis 3) Mixed cellularity 4) Lymphocytic depletion
Photo by Thomas Loire

HIV

Aids
HIV- still around don't forget to consider it as a possible differential.

https://www.khanacademy.org/science/health-and-medicine/infectious-diseases...

https://www.aids.gov/
Photo by unicefukraine

Immunodeficieny

Disorder
Wiskott-Aldrich Syndrome
*thromboctyopenia
*Eczema
*immunodeficiency of selective functions of B lymphocytes and T lymphocytes
*Xlinked recessive inheritance

Photo by tranchis

Blood Type

important to know for transfusion
https://www.khanacademy.org/test-prep/mcat/organ-systems/hematologic-system...


Type and cross necessary- think about treating immune disorders with immune components found in the blood.
Remember Kawasaki disease!
Photo by dsevilla

Anaphylaxis

worst nightmare with blood transfusion
Photo by emmajanehw

Untitled Slide

Hannah Cilli

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