HEMATOLOGIC DISORDERS HEMATOLOGY The hematologic or hematopoietic system includes the blood, blood vessels, and blood forming

g organs ( bone marrow, spleen, liver, lymph nodes, and thymus gland). Major function of blood is to carry necessary materials ( oxygen, nutrients ) to cells and to remove C ! system and metabolic waste products. "t also plays a role in hormone transport, inflammatory and immune responses, temperature regulation, fluid#electrolyte balance, and acid#base balance. THREE BROAD FUNCTIONS OF BLOOD : 1. Transportation Respirator $ transport of gases by the %&C N!triti"e $ transport of digested nutrients from the '"T to the different cells of the body E#$retor $ transport of metabolic wastes to the (idneys and excreted as urine %. Re&!'ation )ormones and other molecules that help regulate metabolism are also carried in the blood Thermoregulation *rotection &lood clotting +eu(ocytes COM(ONENTS OF THE BLOOD : 1. ('as)a %. For)e* E'e)ents ,rythrocytes or %&C +eu(ocytes or -&C Thrombocytes or *latelets BLOOD .verage volume is / $ 0 liters or approximately 0 1uarts p) is 2.3/ $ 2.4/ .rterial blood is usually bright red in color compared to venous blood which has a dar(er color, due primarily to the large concentration of oxyhemoglobin found in arterial blood

FORMED ELEMENTS OF THE BLOOD Er t/ro$ tes .lso called %ed &lood Cells or %&C>s =unction primarily to ferry xygen in the blood to all cells in the body .lso transports Carbon dioxide out of the body +ifespan of <!8 days only )emoglobin in the %&C binds with the xygen as it is transported in the blood =emale ? <! $ <0 g5<88ml Male ? <3 $ <@ g5<88ml Nor)a' RBC $o!nt? about 4 $ 0 million5mmA He)ato$rit 0HCT, $ percentage of %&C per given volume of blood and is an important indicator of the xygen#carrying capacity of the blood =emale ? 32 $ 4@9 Male ? 4/ $ /!9 Le!1o$ tes .lso called -hite &lood Cells or -&C>s .verage value ? 4,888 $ <<,888 5 mmA *rotects the body against any damage .re able to slip in and out of the blood vessels by ameboid fashion $ in a process called diapedesis -hen mobiliBed, the body speeds up production which usually indicates the presence of infection in the body Le!1o$ tosis $ total -&C count above <<,888 5 mmA Le!1openia $ an abnormally low -&C count T pes: 1. Gran!'o$ tes Ne!trop/i's .Basop/i's Eosinop/i's %. A&ran!'o$ tes

Lymphocytes

-Monocytes

a.) Plasma - fluid portion of the blood

Contains : proteins 5 albumin clotting factors waste products fibrinogen electrolytes nutrients

+., Ce''!'ar Co)ponents 1. +eu(ocytes (-&C) !. ,rythrocyte (%&C) 3. Thrombocyte (*latelets) He)atopoiesis- occurs in the bone marrow ( pelvis, ribs, vertebrae and sternum. E#tra)e*!''ar He)atopoiesis. the liver and the spleen produces blood cells (LASMA The li1uid part of the blood6approximately 789 water .lso contains nutrients, ions (salts, primarily :a), respiratory gases, hormones, plasma proteins, antibodies and various wastes and products of cellular metabolism *+.;M. *% T,":; $ the most abundant solutes in the plasma T/ree T pes <. .lbumin !. 'lobulin 3. =ibrinogen 4.

Ce''s o2 t/e I))!ne S ste) L )p/o$ tes +ymphocytes are created in the bone marrow and migrate to the Thymus where they mature .fter becoming immunocompetent, the & C T cells transfer to the lymph nodes C spleen T pes 1.B ' )p/o$ tes or B $e''s - produces antibodies to incapacitate the antigen 2.T ' )p/o$ tes or T Ce''s - attac(s antigens directly Ma$rop/a&es +iterally means D&ig ,atersE .rise from monocytes formed in the bone marrow Major role ? to engulf foreign particles Ce''!'ar 0Ce''.Me*iate*, I))!ne Response T - Ce''s %esponds directly to antigens -ill destroy target cells thru secretions of +ympho(ines and *erforin ( DFiss of GeathE) which is inserted to the cell membrane, shortly after that, the target cell ruptures They have a? +icense to F"++ +icense to ),+* +icense to ;uppress T/ree t pes : 3i''er T Ce''s $ binds to the surface of invading cells, disrupt the cell membrane C destroy it by altering it>s environment He'per T $e''s $ helps to stimulate the & Cells to mature into *lasma Cells which synthetiBe C secrete immunoglobulins (.ntibodies)

 S!ppressor T Ce''s $ %educes the

)umoral response

K transported to bone marrow via T%.:;=,%": C reclaimed for new )gb production ERYTHRO(OIESIS# %&C production Re6!ire)ents : a. ,rythropoietin b. "ron c. =olic .cid d. Litamin &0, Litamin &<! e. Litamin C 77 Li"er an* Sp'een. 'raveyard of the %&C He)ostasis 0B'oo* C'ottin&,

.T/ree Ma8or (/ases

H!)ora' 0Anti+o* .Me*iate*, I))!ne Response B Ce''s Mat!res into ('as)a Ce''s responsi+'e 2or Antibody production 4 C'asses o2 I))!no&'o+!'ins 0MADGE, : I))!no&'o+!'in M 0I&M, <st immunoglobulin produced in an immune responsepresent in plasma, too big to cross membrane barriers I))!no&'o+!'in A 0I&A, ;ound in body secretions li(e saliva, tears, mucus, bile, mil( C colostrum I))!no&'o+!'in D 0I&D, *resent only in the plasma C is always attached to the & Cell I))!no&'o+i'in G 0I&G, @89 of circulating antibodies Can cross the placenta and provide passive immunity *resent in all body fluids I))!no&'o+!'in E 0I&E, %esponsible for .llergic C hypersensitivity reactions ;timulates Mast cells C &asophils to release )istamine which mediates inflammation C the allergic response T/ro)+o$ tes .lso called *latelets .verage value ? !/8,888 $ 4/8,888 5 mmA +ives for about / $ <8 days "mportant in blood clotting He)atopoiesis 0B'oo* Ce'' For)ation, ccurs in the %ed &one Marrow, chiefly in flat bones li(e ;(ull, ribs, pelvis, sternum and proximal epiphyses of the humerus and femur ,rythropoiesis $ %&C production, is a very active process %&C are continuously being destroyed by the liver C spleen %&C>s have a lifespan of <!8 days .s %&C>s are destroyed, iron is recycled to the bone marrow for use in the formation of new %&C>s ,rythropoietin $ secreted by the (idneys C released when blood levels of xygen begins to decline for any reason6 which stimulates the %ed &one Marrow to pro*!$e )ore RBC5s ERYTHROCYTES H destruction # mature cells removed chiefly by spleen C liver I &"+"%J&": K byproduct of )gb released when %&C>s destroyed I "% : K freed from )gb during bilirubin formation

1. ('ate'et ('!& For)ation *latelets adhere and stic( to vessel lining that are damaged forming a *latelet *lug or -hite Thrombus *latelets release chemicals to attract more platelets to the injured site %. 9as$!'ar Spas)s *latelets release ;erotonin causing spasms of the blood vessel, constricting it C decreasing blood flow :. Coa&!'ation or B'oo* C'ottin& Thromboplastin is released by damaged cells plasma Clotting =actors form an activator that triggers the Clotting Cascade a &lood Clot is formed ;erum is s1ueeBed out within the hour pulling the ruptured edges together

('as)a C'ottin& Fa$tors I Fi+rino&en II (rot/ro)+in III Tiss!e T/ro)+op'astin I9 Ca'$i!) 9 (roa$e'erin 9II (ro$on"ertin 9III Anti/e)op/i'i$ Fa$tor I; C/rist)as Fa$tor ; St!art - (ro<er Fa$tor ;I ('as)a T/ro)+op'astin Ante$e*ent ;II Ha&e)an Fa$tor ;III Fi+rin Sta+i'i=in& Fa$tor Co)pati+'e B'oo* T pes ANEMIA Conditions in which the number of %&C>s or amount of hemoglobin is lower than normal leads to hypoxia and ischemia C'assi2i$ations o2 Ane)ia A$$or*in& to Etio'o&

Iron De2i$ien$ Ane)ia most common type of anemia "ron stores are depleted, resulting in a decreased supply of iron for the manufacture of hemoglobin in %&C>s Commonly results from +'oo* 'oss> in$rease* )eta+o'i$

*e)an*s> s n*ro)es o2 &astrointestina' )a'a+sorption> an* *ietar ina*e6!a$

cause ? inade1uate absorption or excessive loss of iron &leeding $ principal cause in adults Legetarian diets Litamin C $ increases iron absorption Assess)ent Fin*in&s

#fatigue, dyspnea, palpitatations C diBBiness, pallor, brittle hair C nails, pica, &'ossitis> $/ei'osis> 1oi'on $/ia Mild cases $ asymptomatic

C/ei'osis La+orator 2in*in&s : RBC5s are s)a'' ? )i$ro$ ti$ an* pa'e hemoglobin C hematocrit serum iron C ferritin N!rsin& Inter"entions 1. I*enti2 t/e $a!se %. Monitor S?S# o2 +'ee*in& - stoo'> !rine an* GI $ontents :. (ro"i*e rest @. Gi"e iron preparations 0 A - 1% )ont/s , #=errous ;ulfate, 'luconate, =umarate a. always give after meals or snac(s b. dilute li1uid preps and give thru straw c. give with orange juice (Litamin C enhances absorption) d. warn clients the stool will become blac( and can cause constipation 4. For $'ients <it/ poor a+sorption or $ontin!o!s +'oo* 'oss ."M or "L of "ron Gextran a. Jse < needle to withdraw and another for injection b. Jse B#trac( method c. don>t massage but encourage ambulation d. usually, deep "M at buttoc(s A. Gi"e *ietar tea$/in& - 'i"er> )eats> n!ts> e&& o'1> s/e''2is/> 'e&!)es> et$. B. In$rease inta1e o2 ro!&/a&e an* 2'!i*s to pre"ent $onstipation. (erni$io!s Ane)ia Litamin &<! Geficiency .nemia caused by inade1uate Lit. &<! inta(e or deficiency in intrinsic factor Lit. &<! combines with intrinsic factor so it can be absorbed in the ileum into the bloodstream the result is abnormally large erythrocytes and hypochlorhydria ( a deficiency of hydrochloric acid in gastric secretions). +ac( of intrinsic factor is caused by gastric mucosal atrophy (possibly due to heredity, prolonged iron deficiency, or an autoimmune disorder), can also result in client who have had a total gastrectomy Jsually occurs in men and women over age /8, with an increase in blue eyed persons. Assess)ent :
Ane)ia. s )pto)s are: Fati&!e> <ea1ness dyspnea paresthesias wt. loss

Measures absorption of radioactive vitamin &<! both before and after parenteral administration of intrinsic factor. Gefinitive test for pernicious anemia. Jsed to detect lac( of intrinsic factor. =asting client is given radioactive vitamin &<! by mouth and nonradioactive vitamin &<! "M to saturate tissue binding sites and to permit some excretion of radioactive vitamin &<! in the urine if it is absorbed. %@.@D /o!r !rine $o''e$tion is obtained6 client is encouraged to drin( fluids. "f indicated, a second stage ;chilling test will be performed < wee( after first stage. =asting client is given radioactive vitamin &<! combined with human intrinsic factor and the test will be repeated.

N!rsin& Inter"entions ? Treat)ent <. Grug Therapy a. Lit. &<! injections monthly for life b. "ron *reparations c. =olic .cid !. Transfusion therapy 3. &ed rest 4. Mouth care /. Gietary teaching 0. Teach about importance of lifelong Litamin &<! therapy He)o' ti$ Ane)ias rate of %&C destruction short life span of %&C '0*G ;ic(le cell anemia Thalassemia G"C Transfussion incompatibilities Si$1'e Ce'' Ane)ia Most common inherited disease among blac( .mericans. .lso found in .rabian, Mediterranean and Caribbean descent )gb ; ( abnormal hemoglobin ), which has reduced oxygen carrying capacity, replaces all or part of the hemoglobin in the %&C>s. +ife span is 0#!8 days instead of <!8, causing hemolytic anemia. Geath often occurs in early adulthood due to occlusion or infection. Guring decreased ! tension, lowered p), dehydration and severe infections, %&C>s change from round to sic(le or crescent shape ;ic(led cells don>t slide thru vessels as normal %&C>s do, causing clumping, thrombosis, arterial obstruction, increased blood viscosity, hemolysis and eventual tissue ischemia and necrosis Si$1'e Ce'' Crisis : Ca!se ? infection, dehydration, fever, cold exposure, hypoxia, strenuous exercise, extreme fatigue or extreme changes in altitude 9ASO.OCCLUSI9E CRISIS: #most common and most painful type of crisis caused by stasis of blood with clumping of the cells in the microcirculation leading to ischemia C infarction. #signs include fever, pain, and tissue engorgement #trreatment hydration, electrolyte replacement, bed rest, broad spectrum antibiotics, transfusions C oxygen therapy. S(LENIC SEEUESTRATION: #+ife # threatening crisis caused by the pooling of blood in the spleen. (from congestion of sic(led

(a'pitations C *i==iness

(a''or Con2!sion inte''e$t!a' 2!n$tion Sore ton&!e

La+ Res!'ts #Gecrease %&C #Gecreased free )ydrochloric acid #+arge %&C 5 Megaloblast #(ositi"e S$/i''in& Test$ definitive test for *ernicious anemia # used to detect lac( of intrinsic factor (ositi"e s$/i''in& test

cells) #signs include profound anemia, hypovolemia, and shoc( #treatment ? blood transfusions and splenectomy A(LASTIC CRISIS: # ccurs infre1uently and is caused by? diminished production of %&C increased destruction of %&C>s triggered by a viral infection or the depletion of folic acid. #signs include profound anemia, pallor, and *.:CMT *,:".. #treatment $ Transfusion of pac(ed %&C>s =re1uent infection esp. with ). influenBae "nfants may have Gactylitis (hand $ foot syndrome) symmetrical painful soft tissue swelling in the hands and feet in the absence of trauma Assess)ent ;igns and symptoms of anemia $ pallor, wea(ness )epatospleenomegaly Gactylitis (;ymmetric swelling of the hands and feet) $ called hand#foot syndrome ther problems ? CL. M" 'rowth retardation $ initial manifestation Gecreased fertility *riapism %ecurrent severe infections MEDICAL MANAGEMENT .. Grug therapy H analgesic5narcotics to control pain .void meperidine (Gemerol) due increased ris( of seiBures in children H antibiotics to control infection. &. &lood transfusions C. )ydration?oral and "L G. &ed rest ,. ;urgery? splenectomy INTER9ENTIONS .dminister ! C &lood Transfusion as %x Maintain ade1uate hydration .void tight clothing that could impair circulation. Feep wounds clean and dry. *rovide bed rest to decrease energy expenditure and oxygen use. ,ncourage patient to eat foods high in calories, C) :, with folic acid supplementation. .nalgesics? .cetaminophen Morphine avoid aspirin as it enhances acidosis,which promotes sic(ling .void anticoagulants( sludging is not due to clotting ). .ntibiotics. .void activities that re1uire so much energy. Feep arms and legs from extreme cold. Gecrease emotional stress. *rovide good s(in care

"nsufficient &#globulin chain synthesis allows large amounts of unstable chains to accumulate *recipitates of alpha chains that form cause %&C>s to be rigid C easily destroyed, leading to severe hemolytic anemia K chronic hypoxia ;(eletal deformities? pathologic fractures )emosiderosis $ excess iron supply, which leads to iron deposits in the organ tissues leading to decreased function

CLINICAL MANIFESTATIONS onset is usually insidious ;x are primarily related to progressive anemia, expansion of marrow cavities of the bone C developmemnt of hemosiderosis ,arly ;x often include progressive pallor, poor feeding C lethargy =urther signs? hemorrhage, bone pain, exercise intolerance, jaundice, C protuberant abdomen /e)osi*erosis o2 t/e e e an* '!n&s G".': ;T"C ,L.+J.T" : Gecrease hemoglobin %&CK increase in number )gb elctrophoresis elevated levels of )g= ( doesn>t hold ! well ) limited amount of )g. Mana&e)ent =re1uent and regular transfusion of pac(ed %&C>s to maintain )gb levels above <8 g5d+ "ron chelation therapy with deferoxamine (Gesferal) $ reduces toxic effects of excess iron C increases iron excretion thru urine C feces ;plenectomy ;upportive management of symptoms &one marrow transplant *rognosis and ;urvival rate is poor because of no (nown cure ften fatal in late adolescence or early adulthood Co)p'i$ations Sp'eno)e&a' Gro<t/ retar*ation in t/e se$on* *e$a*e En*o$rine a+nor)a'ities : delayed development of secondary sex characteristics $ most boys fail to undergo puberty, girls $ menstruation problems GM $ due to iron deposits in the pancreas )ypermetabolic rates S1e'eta' $o)p'i$ations =rontal C parietal bossing (,nlargement) Maxillary hypertrophy $ leading to occlusion *remature closure of epiphyses of long bones steoporosis C pathologic fractures Cardiac problems? pericarditis C C)= $ usual cause of death Co)p'i$ations 'allbladder disease 'allstones that often re1uire surgery ;(in $ bronBe pigmentation caused by iron deposits in the dermis +eg ulcers ERYTHROBLASTOSIS FETALIS R/ In$o)pati+i'it

THALASSEMIA MAFOR 0Coo'e 5s ane)ia, & # thalassemia refers to an inherited hemolytic anemia, characteriBed by reduction or absence of the &#globulin chain in )gb synthesis =ragile %&C C short life span .utosomal recessive pattern of inheritance

Gestruction of %&Cs that result from .g#.b rxn CharacteriBed by hemolytic anemia or hyperbilirubinemia *ossibly caused by %h incompatibility between the mother C the fetus (.g C .b reaction)

"mplement phototherapy or exchange transfusion.

ERYTHROBLASTOSIS FETALIS ;ensitiBation of %h (#) woman by transfusion of %h (N) blood ;ensitiBation of %h (#) woman by presence of %h (N) %&Cs from her fetus conceived with %h (N) man .pproximately 0/9 of infants conceived by this combination of parents will be %h (N) Mother is sensitiBed by passage of %h (N) %&Cs thru placenta, either during pregnancy (brea(5lea( in membrane) or at the time of separation of the placenta after delivery. RH INCOM(ATIBILTY FIRST (REGNANCY . )ot/er )a +e$o)e sensiti=e*> +a+ rare' a22e$te* INDIRECT COOMB5S TEST # Tests for anti#%h(N) .b in mother>s circulation # performed during pregnancy at first visit C again about !@ wee(>s gestation. %,;J+T;? # "f (#) at !@ wee(s, a small dose of (Micro%hogam) is given prophylactically to prevent sensitiBation in the 3rd trimester. # %hogam may also be given after !nd trimester amniocentesis # "f (N), levels are titrated to determine potential effects on the fetus DIRECT COOMBS5 TEST # Tests done on the cord blood at delivery to determine presence of (N) .b on fetal %&Cs %,;J+T; # "f both indirect C direct Coombs> test is :,'.T"L, C infant is %h(N)? # :,'.T"L,? :o formation of .nti#%h (N) .b # %hogam (%hoOGP human immune globulin is given to the %h(#) mother to prevent development of anti#%h(N) .b as the result of sensitiBation from present or just terminated pregnancy. "n each pregnancy, an %h(#) mother who carries an %h (N) fetus receives %hogam if both the mother and infant is (#) to both direct C indirect Coombs> test. "f mother is has been sensitiBed? anti#%h(N) .b are present # %hogam is not indicated %hogam must be injected into unsensitiBed mother>s system within 2! hours of delivery of %h(N) infant ERYTHROBLASTOSIS FETALIS CLINICAL FINDINGS .nemia Qaundice that develops rapidly after birth and before !4 hours or that occurs within !4 # 30 hours ,nlarged placenta ,dema .scites NURSING INTER9ENTIONS Getermine blood type and %h early in pregnancy. Getermine results of direct Coomb>s test early in pregnancy C again at !@ wee(>s. Getermine results of direct Coomb>s test on cord blood. # type C %h, )gb, )ct

NURSING INTER9ENTIONS .dminister %h8 (G) immune globulin to the mother during the first 2! hrs. after delivery if the %h(#) mother delivers an %h (N) fetus but remains unsensitiBed .ssist with exchange transfusion as prescribed. The baby undergoes transfusion of blood to stop the destruction of the baby>s %&C # the transfused blood is replaced with the baby>s own blood gradually %eassure the mother that the newborn will suffer no untoward effects from the condition MYELO(ROLIFERATI9E DISORDER (OLYCYTHEMIA 9ERA Jnderlying cause is un(nown )yperplasia of all bone marrow elements H increase %&C mass H increase blood volume viscosity H decrease marrow iron reserve H ;plenomegaly ASSESSMENT %eddish purple hue of s(in C mucosa, pruritus ;plenomegaly, hepatomegaly ,pigastric discomfort, abdominal discomfort *ainful fingers C toes from paresthesias .ltered mentation -ea(ness, fatigue, night sweats, bleeding tendency )yperuricemia $ from increased %&G formation and destruction D; TESTS C&C & :, M.%% - .;*"%.T" : C &iopsy MANAGEMENT HY(ER9ISCOSITY K phlebotomy R intervals determined by C&C results to decrease %&C mass Kgenerally !/8#/88ml removal R a time HY(ER(LASIA K myelosuppressive therapy, K generally using hydroxyurea or "L radioactive phosphorus (3!*), biologic response modifier, ie alpha interferon HY(ERURICEMIAK allupurinol (Syloprim) (RURITUS K antihistamines (cimitidine), low dose acetyl salicylic acid6 certain anti# depressants (paroxetin), phototherapy, cholestyramine INTER9ENTION ,ncourage5assist ambulation .ssess for early ;5;x of thromboembolic complications ? swelling of limbs, increased warmth, pain Monitor C&C C assist with phlebotomy as ordered (atient E*!$ation ,ducate about ris( of thrombosis6 encourage patient to maintain normal activity pattern C avoid long periods of rest .void hot showers %eport R regular intervals for follow up blood DISORDERS OF (LATELETS an* CLOTTING MECHANISM HEMO(HILIA )ereditary coagulation defect, usually transmitted to affected male by female carrier

through sex $ lin(ed recessive gene, resulting in prolonged clotting time. Most common type is )emophilia . or Classic )emophilia # factor L""" deficiency (called .ntihemophilic =actor 5 .)=) )emophilia & or Christmas Gisease $ factor "T deficiency (called the Christmas =actor) Male inherits hemophilia from their mothers, and females inherit the carrier status from their fathers. =ound predominantly, but not exclusive, in male offsprings &leeding occurs due to impaired ability to form fibrin clot

/e)orr/a&i$ +!''ae> a$ra' $ anosis> 2o$a' &an&rene in s1in D# Tests: mar(ed decrease of blood platelets low levels of fibrinogen C other clotting factors prolonged prothrombin C partial thromboplastin times C abnormal erythrocyte morphologic characteristics N!rsin& Inter"entions ? Treat)ent <. The objective of treatment is to determine the underlying cause of G"C and provide treatment for it. !. %eplacement therapy of the coagulation factors is achieved by transfusion of fresh froBen plasma. Cryoprecipitates may also be used if fibrinogen is significantly decreased. *latelet transfusions if platelets are diminished 3. )eparin, a medication used to prevent thrombosis, is sometimes used in combination with replacement therapy. ( still controversial ) 4. *revent further injury a. avoid "M injections b. apply pressure to bleeding sites c. turn patient fre1uently and gently d. provide mouth care $ soft bristled toothbrush /. Teach patient the importance of avoiding aspirin. IDIO(ATHIC THROMBOCYTO(ENIA (UR(URA "ncreased destruction of platelets with resultant platelet count of less that <88,8885mm3 characteriBed by petechiae and ecchymoses of the s(in. ,xact cause un(nown6 may be autoimmune. ;pleen is the site for destruction of platelets often triggered by J%T" or Childhood communicable disease $ Measles C chic(enpox

ASSESSMENT .bnormal bleeding in response to trauma or surgery. (muscles5joints) Qoint bleeding causing pain, tenderness, swelling, and limited range of motion. Tendency to bruise easily. ,pistaxis )emarthrosis (bleeding in joints causing pain, swelling and limited movement) IM(LEMENTATION .dminister factor L""" concentrate. Monitor for bleeding and maintain bleeding precautions. Monitor for joint pain6 "MM &"+"S, the affected extremity if joint pains occur. Monitor urine for hematuria. "nstruct the parents regarding activities for the child, emphasiBing the avoidance of contact sports. "nstruct the parents on how to control bleeding (direct5indirect pressure) GGL.* (Gesmopressin) $ promotes the release of =actor L""" in hemophilia . Jse soft toothbrush and point out need for regular dental chec(ups %efer to :ational )emophilia .ssociation ,mphasiBe avoidance of .spirin *rovide diet information as excess weight places further stress on joints IM(LEMENTATION R . Rest I . I))o+i'i=e C . Co'* Co)press E . E'e"ate Dissi)enate* Intra"as$!'ar Coa&!'ation G"C is a disorder of diffuse activation of the clotting cascade that results in depletion of clotting factors in the blood. occurs when the blood clotting mechanisms are activated all over the body instead of being localiBed to an area of injury. grave coagulopathy resulting from overstimulation of clotting C anticlotting processess in response to disease C injury ;mall blood clots form throughout the body, and eventually the blood clotting factors are used up and not available to form clots at sites of tissue injury. Clot # dissolving mechanisms are also increased stimulated by many factors including infection in the blood C severe tissue injury $ burns and head injury, reactions to blood transfusions, carcinomas and obstetrical complications such as retained placenta after delivery. ASSESSMENT p!rp!ra on 'o<er e#tre)ities C a+*o)en

ASSESSMENT: *etechiae ,cchymosis &lood in any body secretions, bleeding form mucous membranes, nosebleeds. Gecreased platelet count .nemia easy bruising blood in stool or urine C&C reveals platelet count below !8,8885mm3 &one marrow aspiration done to rule out leu(emia MEDICAL MANAGEMENT: Grug therapy? *rednisone $ decreases anti#platelet antibodies (monitor for infection) "L"' ("ntravenous "mmune 'lobulin) $ helps to effectively increase platelet count .nti#G .ntibody $ one dose treatment 'iven to pt>s < year but less than <7 years old :ormal -&C and hemoglobin no active bleeding present no concurrent infection Giphenhydramine and hydrocortisine are made ready for possible allergic reactions to the medication *latelet transfusion ;plenectomy Inter"ention *revent, control and minimiBe bleeding. *revent bruising *rovide support to client and be sensitive to change in body image. *rotect from infection. .dminister analgesics (acetaminophen) as ordered6 avoid aspirin.

administer meds orally, rectally, or ".L. rather than ".M.