NSG213a
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| Immune disorder where there is a low level of antibodies, manifested by recurrent infections between infancy and adulthood. | CVID Common variable immunodeficiency
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| Cause of CVID? | Unknown- may affect one or more than one family member. Sign is decrease in immunoglobulins
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| Areas of infection with CVID? | *Lungs damage can be permanant *Ears *Sinuses *Join inflammation *stomach/bowel disorder *increase r/f cancer
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| TX for a patient with CVID? | *Immunoglobulin therapy
*med for illness
*postural drainage of the lungs
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| Group of rare, life threatening diseases present at birth. Little or no immune system. Absence of __ cell (humoral) and __ cell (cell mediated) immunity. | SCID Severe combined immunodeficiency
T and B
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| Known as boy in the bubble syndrome | SCID
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| Cause of SCID? | Inherited, many genetic defects an cause; reduced ability of white cells to fight infection. X-linked or autosomal recessive disorder. Affects boys/girls
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| Disease? X linked or autosomal recessive disorder. Inherited. | SCID
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| S/S of SCID? When do symptoms arise? | *Severe pneumonias *meningitis *sepsis *chronic skin infections *yeast of mouth/diaper area *diarrhea *liver infections
**S/S arise within first year**
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| What tx would you expect for a patient with SCID? | *hematopoietic stem cell transplan *IV IG infusion, til bone marrow or stem cell transplant *pneumocystitis carinii pneumonia ther. *bone marrow trans *immune boost meds *gene therapy
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| Best therapy for SCID? | Compatible Bone marrow transplant
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| Wiskot aldrich syndrome (WAS) what is it? | Syndrome where there is a primary immune deficiency involving both T and B lymphocytes. Plateles affected as well. X linked
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| Infant girl is admitted with Wiskott Aldrich syndrome. In what way is this treated differently in a female than a male? | A female won't have this. It is X-linked, a male only disease.
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| S/S of WAS? | *increased bleeding (thrombocytopenia) Hallmark: petechia, bloody stool, bleeding gums, prolonged nose bleed, brain hemorrhage
*Recurrent bacterial, viral, fungal infection esp respiratory tract
*exzema
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| Recurrent infections commonly seen in WAS? | *Respiratory tract *sepsis *meningitis *recurrent Herpes Simplex *Pneumocystitis carinii
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| An older male with WAS may exhibit these symptoms... | *automimmune-like* *an anemia caused by antibodies which destroy RBC's *FUO with no cause *swollen joints *kidney inflamm. *Gi s/s Diarrhea *arterial infl. vasculitis
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| Malignancies involved in WAS | *involves lymphocytes; lymphoma and leukemia
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| Located on the short arm of the X chromosome. X linked recessive disorder, mutations between families are unique, severity varies | WAS
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| How is WAS diagnosed? | platelet count and cell size. Serum antibodies, skin tests to view T-Lymphocyte function.
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| Tx for WAS? | *prophylactic use of IVIG *Bone marrow transp *Iron replacement *Tx infections *vaccines *bath oils *steroid creams *assess food allergy *platelet transfusions
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| What is the only cure for WAS? | Bone marrow or cord blood stem cell transplantation. Possible splenectomy to correct thrombocytopenia
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| First immunodeficiency disease ever identified as X-linked | Agammaglobulinemia (BRutons or congenital)
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| Disease in which there is an inability to produce antibodies that make up gamma globulins in the plasma. failure of pre-bplymphocytes to mature into b-lymphocytes that produce antibodies. | X-linked agammaglobulinemia
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| child cannot fight off bacterial infections and some viral infections | X linked agammaglobulinemia
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| S/S of x linked agammaglobulinemia in first 6-8 mos-3-5 yrs. | First 6-8 mos; sinusitis, rhinitis, pyoderma, conjunctivitis, osteomyelitis, meningitis, sepsis, bronchitis, pneumonia, GI infection, Viral infection: hepatitis, polio, enterovirus
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| S/S of what disease? Growth failure, absence of tonsils/adenoids,joint disease,autoimmune hemolytic anemia, glomerulonephritis, neutropenia, dermatomyositis, cancers | x-link agammaglob
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| Tx for x-linked agammaglob | antibody replacement, infection mgmt, avoidance of live viral vaccines
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| prognosis of x-linked agammaglob without tx? | Death at early age without gamma glob RX, With good med mgmt they may live into adult years.
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| Most childhood cancers arise from what layer? | embryonic mesodermal layer; lesser amount from the ectodermal layer
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| Childhood cancer presents in the deeper organs, making it different from adult cancers. Examples of childhood cancer presentations? | *Compression (tumor)
*Obstruction
*altered CBC
*abnormal secretion of hormones
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| S/S of childhood cancers | *wt loss *anorexia *fever without cause *pallor *NV *fatigue *masses/swollen body parts *visual disturbance *bruising/petechiae *HA without cause *unsteady gait/limp *cough/dyspnea
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| Types of childhood leukemia? | *Acute lymphocytic leukemia (accounts for 3 out of 4 cases.
*Chronic lympho leukemia (rare)
*acute myelogenous accounts for bulk of the remaining cases.
*chronic myelogenous- rare
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| This type of leukemia starts from the lymphoid cells in the bone marrow. | ALL (acute lymphocytic leukemia)aka acute lymphoblastic
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| This leukemia starts from the cells that form white blood cells (other than lymphocytes) | AML- acute myelogenous/aka acute myeloid, acute myelocytic, acute non-lymphocytic leukemia
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| Rare leukemia in children r/t CML philadelphia chromosome | Chronic myelogenous
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| If a child with Chronic myelogenous leukemia goes into remission after treatment, what additional treatment would be suggested? | Stem cell transplant
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| Cause of fatigue and paleness of skin in presence of leukemia? | anemia
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| Cause of infections and fever in presence of leukemia? | Fever of unknown origins, often r/t infection, which may not improve with antibio. R/T a lack of normal WBC's particularly mature granulocytes.
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| Cause of bleeding/bruising of skin in presence of leukemia? | r/t bleed from tiny blood vessels
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| Bone or joint pain causes in leukemia? | R/T buldup of leukemia cells near surface of bone or inside joint
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| What causes swelling of abdomen in leukemia? | cells may collect in liver and spleen causing enlargement.
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| What causes the loss of appetite/weight loss in leukemia? | Enlargement of liver and spleen causing them to place pressure on the stomach.
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| Enlarged lymph nodes in children, are most often a sign of leukemia. T/F? | False, they are often a sign of infection only.
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| Enlargement of __________ can be the cause of coughing. | Thymus or lymph nodes in chest press on trachea.
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| Leukemia Cause of swelling in face and neck? | superia vena cava is next to the thymus, when thymus enlarges, it can press on SVC causing blood to back up. EMERGENCY
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| In the presence of superior vena cava compression in a child with leukemia, what signs beyond swelling of the face and arms may be seen? | bluish-red coloration of head,arms and upper chest. can also cause trouble breathing and change in consciousness- affecting brain
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| headache, concentration problems, weakness, seizure, vomiting, balance problems, blurred vision can be systems of what complication in leukemia? | Central system involved leukemia
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| Collection of AML cells under skin or other parts of the body are called.... | chloroma or granulocytic sarcoma
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| When symptoms of extreme tiredness, weakness, slurred speech occurs, what is the cause? | high numbers of leukemia cells, making the blood too thick interfering with small vessel brain circulation.
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| Diagnostics in leukemia- CBC is done to reveal? | Determines how many of each type of blood cell is present in the blood. Blood smear also done for microscopic analysis
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| Many of the WBC found in the blood in leukemia will be ______ which is found only in the bone marrow. | Bone marrow
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| Bone marrow biopsies are taken from the back of the pelvic bone, but may also be taken from this area. | Sternum or other bones.
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| Why would a lumbar puncture be done in leukemia? | Determines cells present in the CSF, and also can be a route to admin chemo drugs to prevent or tx spread of leukemia to the spinal cord and brain.
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| Lymph node biopsy is important in dx lymphomas but-- | is rarely needed for children with leukemias
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| What 3 chemo phases are used in tx of children with acute lymphocytic leukemia? | 1. induction
2. consolidation/intensification
3. maintenance
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