???????????????????????????????????????????? largest
reticuloendothelial organ(?) fromderm(?) ligaments of spleen(?)
Vascular most of spleens blood derives from(?) three main branches
of the celiac artery(?) accessory spleen Present in up to (?%)
Spleen size & weight (?) Splenomegaly,Hypersplenism
erythrocyte's, neutrophil & Platelets, life span (?)
splenectomy should be delayed if possible to at least ??? age AMINI
MD
Slide 5
Embryology & anatomy Spleen is the largest
reticuloendothelial organ in the body From the primitive mesoderm
Evident in the fifth week of gestation in an 8 mm embryo The most
common variation of splenic embryology is the accessory spleen.
Present in up to 20% of the population AMINI MD
Slide 6
Ligaments of the spleen (splenocolic ligament), to the colon
(phrenosplenic ligament),to the diaphragm (splenorenal ligament) to
the kidney, adrenal, and tail of the pancreas (gastrosplenic
ligament),to the stomach The gastrosplenic ligament contains the
short gastric vessels; the remaining ligaments are avascular AMINI
MD
Slide 7
Anatomy cont. the tail of the pancreas has been demonstrated to
lie within one cm of the splenic hilum 75% of the time and in 25%
of patients actually to abut the spleen AMINI MD
Slide 8
ligaments
Slide 9
Slide 10
The spleen derives most of its blood from the splenic artery,
the longest and most tortuous of the three main branches of the
celiac artery AMINI MD
Slide 11
Slide 12
Accessory spleens Accessory spleens Present in up to 20% of the
population Over 80% in the hilum and vascular pedicle Other
locations in descending order gastrocolic ligament, greater
omentum, stomach's greater curve, splenocolic ligament, small and
large bowel mesentery, left broad ligament in women, and the left
spermatic cord in men AMINI MD
Slide 13
Locations of accessory spleens AMINI MD
Slide 14
splenic artery & vein The distributed type :is the most
common (70%) and is distinguished by a short trunk with many long
branches. The less common magistral type of splenic artery (30%)
has a long main trunk dividing near the hilum into short terminal
branches. The splenic vein joins the superior mesenteric vein to
form the portal vein and accommodates the major venous drainage of
the spleen. AMINI MD
Slide 15
Spleen size The average adult spleen is 7 to 11cm in length and
weighs 150 g (range 70 to 250 g). palpable below the left costal
margin are at least double normal size, with an estimated weight of
750 g Spleen size and weight both diminishing in the elderly AMINI
MD
Slide 16
Definitions, function The spleen can cause significant
hematologic disturbance if it becomes hyper functioning (
hypersplenism) or hypertrophied ( splenomegaly) AMINI MD
Slide 17
Definitions Splenomegaly:( ?>1 kg or a pole-to-pole length
of ?>15 cm) Hypersplenism :the presence of one or more
cytopenias with normally functioning bone marrow.(e.g., hemolytic
anemia ) AMINI MD
Slide 18
Massive splenomegalySplenomegalyNL SpleenSpleen >1 kg500
g70250(150) gmWt >22 cm and / or 15 cm.711 cmLength AMINI
MD
Slide 19
immunologic role spleen plays a key immunologic role in defense
against a number of organisms, particularly encapsulated bacteria
AMINI MD
Slide 20
physiology The red pulp dynamic filtration system, enabling
macrophages to remove microorganisms, cellular debris, antigen-
antibody complexes, and senescent erythrocytes from the
circulation. white pulp nodules that are 9 mg/dL, with intensive
parenteral chelation therapy with deferoxa">
Thalassemia treatment Thalassemia Treatment involves
transfusions to maintain a Hb >9 mg/dL, with intensive
parenteral chelation therapy with deferoxamine. AMINI MD
Slide 57
Thalassemia major Other characteristics are intractable leg
ulcers, head enlargement, frequent infections, the need for
periodic blood transfusions. Untreated individuals usually die in
late infancy or early childhood from severe anemia. Splenectomy is
indicated for patients with excessive transfusion (>200 mL/kg
per year) AMINI MD
Slide 58
Thalassemia patients are at high risk for pulmonary
hypertension after splenectomy AMINI MD
Slide 59
INDICATIONS FOR SPLENECTOMY Splenic rupture (trauma) Red blood
cell disorders and hemoglobino- pathies. White blood cell
disorders, Platelet disorders, Bone marrow disorders
(myeloproliferative disorders), Cysts and tumors, Infections and
abscesses, Storage diseases and infiltrative disorders,
miscellaneous disorders and lesions AMINI MD
Slide 60
INDICATIONS FOR SPLENECTOMY Splenic rupture (trauma) Red blood
cell disorders and hemoglobino- pathies. White blood cell
disorders, Platelet disorders, Bone marrow disorders
(myeloproliferative disorders), Cysts and tumors, Infections and
abscesses, Storage diseases and infiltrative disorders,
miscellaneous disorders and lesions AMINI MD
Slide 61
Lymphomas Hodgkins Disease: Hodgkins disease is a malignant
lymphoma that usually affects young adults in their 20s and 30s.
Rarely, patients present with constitutional symptoms such as night
sweats, weight loss, and pruritus but, more typically, asymptomatic
lymphadenopathy usually involving the cervical nodes
Slide 62
staging Hodgkins disease Staging methods have evolved to
include imaging techniques computed tomography (CT),
18F-fluorodeoxyglucose positron emission tomography (FDG-PET), and
lymphangiography
Slide 63
staging laparotomy Hodgkins disease Historically, included
splenectomy liver biopsy splenic hilar lymphadenectomy
retroperitoneal node biopsy biopsy of a hepatoduodenal node and
oophoropexy in premenopausal women common in stages I and II
disease to rule out splenic or subdiaphragmatic involvement.
Slide 64
Early-stage Hodgkins disease is often cured with radiation
therapy alone. Laparotomy is no longer indicated for patients
likely to relapse, those with evidence of intra-abdominal
involvement on imaging, and those with B symptoms. These patients
should receive systemic chemotherapy
Slide 65
Non-Hodgkins Lymphoma (NHL) Splenomegaly or hypersplenism is
common Splenectomy is indicated for NHL patients with massive
splenomegaly leading to abdominal pain, early satiety, and Fullness
also indicated in hypersplenism (anemia, neutropenia, and
thrombocytopenia)
Slide 66
NHL Splenectomy may also be instrumental in the diagnosis and
staging of patients with isolated splenic disease. The most common
primary splenic neoplasm is NHL 50% to 80% of patients with NHL
have involvement of the spleen but less than 1% of patients present
with splenomegaly without lymphadenopathy Most patients have
low-grade NHL, with frequent involvement of the splenic hilar lymph
nodes, extrahilar nodes, bone marrow, or liver.Approximately 75% of
these patients have clinically apparent hypersplenism
Slide 67
NHL In patients with spleen-predominant features, survival is
significantly improved after splenectomy compared with similar
patients who did not undergo splenectomy
Slide 68
Hairy Cell Leukemia Hairy cell leukemia, a rare disease that
approximately 2% of adult leukemias This disease affects older men
who presents with palpable splenomegaly. Approximately 10% of
patients require no treatment because of the indolent course
Treatment for cytopenias or splenomegaly typically begins with
purine analogue chemotherapy. For more refractory cancers, a
second-line immunotherapy may be instituted
Slide 69
splenectomy in hairy cell leukemia splenectomy in hairy cell
leukemia If chemotherapy or immunotherapy failed in symptomatic
anemia, infections from neutropenia, or hemorrhage from
thrombocytopenia can lead to splenectomy. Patients with diffusely
involved bone marrow without massive splenomegaly are less
responsive to splenectomy. Patients with hairy cell leukemia are
also at a two- to threefold risk for developing other malignancies
(solid tumors)
Slide 70
Chronic lymphocytic leukemia (CLL) is disease of B lymphocytes
characterized by the progressive accumulation of relatively mature
but functionally incompetent lymphocytes. CLL is seen mainly after
the age of 50 Medical treatment is indicated for symptomatic
patients or those exhibiting evidence of rapid disease progression.
Monoclonal antibodies are also used in the treatment of CLL. Bone
marrow transplantation currently offers the only known cure for
CLL. Splenectomy is indicated for patients with refractory
splenomegaly and pancytopenia, which results in improvements in
blood counts in 60% to 70% of patients
Slide 71
Chronic myelogenous leukemia (CML) is a myeloproliferative
disorder & may occur in patients from childhood to old age
characterized by the progressive replacement of normal diploid
elements of the bone marrow with mature-appearing neoplastic
myeloid cells Although CML can be asymptomatic at presentation,
patients commonly present with fever, fatigue, malaise, effects of
pancytopenia (infections, anemia, easy bruising), and occasionally
splenomegaly
Slide 72
CML Bone marrow transplantation is an option but prognosis has
improved dramatically with the advent of recent therapies, making
transplantation less common Symptomatic splenomegaly and
hypersplenism in CML can be effectively treated with splenectomy,
but there does not appear to be a survival benefit when performed
during the early chronic phase. Surgery is therefore reserved for
patients with significant symptoms.
Slide 73
CML the Philadelphia chromosome, is highly associated with
CML
Slide 74
Non-Hematologic Tumors of the Spleen The spleen can also be the
site of metastatic disease, seen in up to 7% of autopsies of cancer
patients. most frequently spread to the spleen are carcinomas of
the breast, lung, and melanoma. Any primary malignancy, however,
can metastasize to the spleen splenectomy may provide palliation
for carefully chosen patients with symptomatic splenic
metastases
Slide 75
white blood cell disorders Splenectomy for white blood cell
disorders can be effective therapy for symptomatic splenomegaly and
hypersplenism, improving some clinical parameters but generally not
altering the course of the underlying disease. Splenectomy is
indicated to improve cytopenias and was shown to be 75% effective
in a combined group of patients who had either CLL or nonmalignant
Hodgkin's disease. AMINI MD
Slide 76
INDICATIONS FOR SPLENECTOMY Splenic rupture (trauma) Red blood
cell disorders and hemoglobino- pathies. White blood cell
disorders, Platelet disorders, Bone marrow disorders
(myeloproliferative disorders), Cysts and tumors, Infections and
abscesses, Storage diseases and infiltrative disorders,
miscellaneous disorders and lesions AMINI MD
Slide 77
Splenectomy in Benign Hematologic Conditions Aged red blood
cells with decreased plasticity (>120 days) become trapped and
destroyed in the spleen. SPLENECTOMY in Benign Hematologic
Conditions Immune (idiopathic) Thrombocytopenic Purpura (ITP), is
characterized by a low platelet count despite normal bone marrow
and the absence of other causes of thrombocytopenia. Autoantibodies
are responsible for the disordered platelet destruction mediated by
the overactivated platelet phagocytosis within the
reticuloendothelial system.
Slide 78
Platelet Disorders IDIOPATHIC (immune)THROMBOCYTOPENIC PURPURA
(ITP) Is an autoimmune disorder Antiplatelet immunoglobulin G
autoantibodies produced in the spleen premature removal of
platelets opsonized by antiplatelet immunoglobulin G
autoantibodies. Characterized by a low platelet count and
mucocutaneous and petechial bleeding & .. AMINI MD
Slide 79
Dif. Dx. Of ITP
Slide 80
ITP presentation purpura Epistaxis gingival bleeding. Less
commonly, gastrointestinal bleeding and hematuria are noted.
Intracerebral hemorrhage is a rare but sometimes fatal
presentation. The diagnosis of ITP involves the exclusion of other
relatively common causes of thrombocytopeniapregnancy, drug-induced
thrombocytopenia (e.g., heparin, quinidine, quinine, sulfonamides),
viral infections, and hypersplenism
Slide 81
Management of ITP Asymptomatic patients with platelet counts
higher than 50,000/mm3 may be observed without further
intervention. Platelet counts of 50,000/mm3 and higher are rarely
associated with clinical sequelae, even with invasive procedures.
Patients with platelet counts, between 30,000 and 50,000/mm3, may
always be observed but with more routine follow-up because they are
at increased risk for progressing to severe thrombocytopenia.
Slide 82
Initial medical treatment is glucocorticoid prednisone, 1 mg/kg
body weight/day) for patients with platelets counts
ITP Of patients treated with steroids, 25% will experience a
complete response. Patients with platelet counts >20,000 /mm3
who remain symptom-free, or who experience minor purpura as their
only symptom, do not require hospitalization
Slide 84
ITP Hospitalization may be required for patients whose
platelets counts remain below 20,000/mm3 with significant mucous
membrane bleeding and is required for those who have
life-threatening hemorrhage. Platelet transfusion is indicated only
for those who experience severe hemorrhage. IV immunoglobulin is
important for the treatment of acute bleeding, in pregnancy, or for
patients being prepared for operation, including splenectomy. The
usual dose is 1 g/kg body weight/day for 2 days.
Slide 85
ITP This dose usually increases the platelet count within 3
days; it also increases the efficacy of platelet transfusions For
patients with severe thrombocytopenia, with counts less than
10,000/mm3 for 6 weeks or longer, those with thrombocytopenia
refractory to glucocorticoid treatment, or those who require toxic
doses of steroid to achieve remission, the treatment of choice is
to proceed to splenectomy
Slide 86
ITP Splenectomy is also the treatment of choice for patients
with incomplete response to glucocorticoid treatment and for
pregnant women in the second trimester of pregnancy who have also
failed steroid treatment or IV Ig therapy with platelet counts less
than 10,000/mm3 without symptoms or less than 30,000/mm3 with
bleeding problems.
Slide 87
ITP It is not necessary to proceed to splenectomy for patients
who have platelet counts higher than 50,000/mm3, have had ITP for
longer than 6 months, are not experiencing bleeding symptoms, and
who are not engaged in high-risk activities. A recent review of
short-term and long-term failure of laparoscopic splenectomy has
reported an overall approximate failure rate of 28% at 5 years
after splenectomy
Slide 88
ITP A systematic review has showed that 72% of patients with
ITP had a complete response to splenectomy. Relapse occurred in a
median of 15% of patients (range, 1% to 51%), with a median
follow-up of 33 months Younger patients had improved responses
Preoperative indium-111 labeled platelet scintigraphy with
platelets sequestered predominantly within the spleen had a
significantly higher response rate than those noted to have hepatic
sequestration
Slide 89
ITP Durable platelet responses are associated with patients who
have platelet counts of 150,000/mm3 by postoperative day 3 or more
than 500,000/mm3 by the postoperative day 10 the long-term failure
rate of laparoscopic splenectomy at approximately 8% and
approximately 44/1000 patient-years of follow-up. Another study has
estimated the complete response of ITP patients postsplenectomy to
be 66%
Slide 90
ITP evaluation for a missed accessory spleen must be undertaken
in patients who experience a relapse. Other treatment options for
these patients include observation of stable nonbleeding patients
with platelet counts higher than 30,000/mm3, long-term
glucocorticoid therapy, and treatment with azathioprine or
cyclophosphamide. Recent evidence regarding thrombopoietin receptor
agonists may offer a novel medical therapy for patients with no
response to steroids, IV immunoglobulin therapy, or
splenectomy.
Slide 91
ITP Gingival bleeding, epistaxis, menorrhagia, hematuria, or
even melena. Platelet >50,000/cc present with incidental
findings; 30,000