The Spleen!

The 1st Page

100

In 350 BC he described the spleen as having a “hot character” that aided in digestion”

Who is Aristotle

200

Writings about this organ in the first century described it as being the source of laughter.

What is The Spleen?

300

This was the organ to which “Hypochondrial and Hysterical Afflictions” was attributed.

What is The Spleen!

400

Although in 1877 the mortality was > 70%, it is the mortality of an elective laparoscopic splenectomy today.

What is <1%

500

Which of the following is NOT produced in significant amounts by the spleen? A. Tuftsin B. Immunoglobulin A (IgA) C. Properdin D. Opsonic antibodies

Ans: B. The Spleen produces opsonins, tuftsin and properidin. Mostly IgM is generated by the lymphoid centers (not IgA).

Pathophys

100

Splenic vein thrombosis (A) commonly presents with isolated esophageal varices (B) is usually accompanied by cirrhosis and portal venous hypertension (C) is usually a consequence of pancreatic pathology (D) should be managed initially with a course of thrombolytic therapy followed by systemic anticoagulation (E) is usually accompanied by hypersplenism and thrombocytopenia

ANS: C. What is Isolated thrombosis of the splenic vein occurs most commonly as a consequence of an underlying pancreatic inflammatory process or from infiltration by a neighboring pancreatic malignancy. The vessel may thus be patent but functionally occluded or actually thrombosed because of stasis, intimal injury from pancreatitis, or by systemic hypercoagulability.

200

Which of the following is the most common etiology of splenic cyst worldwide? A. Bacterial infection B. Trauma C. Parasitic infection D. Congenital anomaly

Ans: C. Parasitic infection (usu Echinococcus) is the most common cause worldwide. Nonparasitic cysts are usually secondary to trauma ⇒ pseudocysts

300

Which of the following splenic ligaments is NOT an avascular plane? A. Gastrosplenic B. Splenocolic C. Phrenosplenic D. Splenorenal

Ans: A. Short gastrics run in the Gastrosplenic ligament.

400

Splenectomy is of proven benefit in the management of the following autoimmune conditions except: (A) Felty’s syndrome (B) immune thrombocytopenic purpura (ITP) (C)cold-agglutinin syndrome (IgM autoimmune hemolytic anemia) (D) warm-agglutinin syndrome (IgG autoimmune hemolytic anemia) (E) systemic lupus erythematosus

ANS: C. Felty’s syndrome, ITP, IgG (warm) autoimmune hemolytic anemia, and systemic lupus erythematosus are acquired autoimmune disorders mediated by an IgG autoantibody against cellular membrane proteins. Splenectomy does not eliminate autoantibody production; it simply ameliorates cell destruction. Hemolysis in IgM autoimmune hemolytic anemia (Cold agglutinin syndrome) is thus mediated by complement and takes place intravascularly rather than extravascularly with clinical manifestations resembling Raynaud’s syndrome. For these reasons splenectomy plays no role in the management of IgM autoimmune hemolytic anemia.

500

DAILY DOUBLE A 65-year-old male with a history of hypertension and diabetes presents to the ER with hematemesis. He claims to have no history of PUD, or significant weight loss. Two weeks ago he underwent debridement and drainage for an infected pancreatic pseudocyst, for which external drains were placed. He also underwent a recent colonoscopy with findings of benign polyps. On physical examination, he is afebrile, with vital signs showing a BP of 90/60 mmHg, HR 120 bpm, RR of 21 breaths/min. Abdominal examination revealed a soft abdomen, two Jackson-Pratt (JP) drains over the left hemiabdomen draining brownish material, slightly tender over the drain site, with a palpable spleen near the umbilicus with normoactive bowel sounds. An NGT was inserted and revealed fresh nonclotting blood. Laboratory examinations showed a hemoglobin of 6.1 g/dL. IVF and blood products given and a CT scan was performed which showed nonenhancing pancreas, no free air, no intraabdominal fluid or abscess but did show splenomegaly and splenic vein thrombosis. An EGD was performed which showed no ulcers, no erosions, but gastric varices were noted with no stigmata of recent bleed. Over the next 2 days, the patient stabilized, but then had another episode of GI bleed. Urgent endoscopy was performed which showed bleeding gastric varices. No attempts at endoscopic therapy were performed. Later that evening, the patient becomes hypotensive with a Hgb of 7 g/dL, BP of 80/60 mmHg, and HR of 120 bpm. What is the therapeutic modality of choice? A. splenectomy and wedge resection of the gastric varices B. subtotal gastrectomy C. splenectomy D. repeat EGD E. angiogram with arterial embolization

Ans: C. Gastric varices indicate left sided (sinistral) hypertension with splenic vein thrombosis likely from the pancreatitis. The treatment is splenectomy, which eliminates splenic artery inflow and venous outflow with immediate reduction of variceal blood flow.

Steroids and Stuff

100

Which of the following proteins is not altered in hereditary spherocytosis (HS)? A. Pyruvate kinase B. Spectrin C. Ankyrin D. Band 3 protein

Ans: A. Pyruvate kinase deficiency can result in chronic hemolytic anemia resulting in chronic congenital hemolytic anemia. Heriditary Spherocytosis results from abnormalities in spectrin, ankyrin and Band 3.

200

Which of the following is appropriate initial therapy in a patient diagnosis with glucose-6-phosphate dehydrogenase (G6PD) deficiency? A. Total splenectomy B. Partial splenectomy C. Exchange transfusion D. Dietary counseling

Ans: D. Certain foods and drugs are known to precipitate a G6PD crisis. Splenectomies are rarely if ever indicated or helpful. A small study of 6 patients with a particular mutation (at exon 10) with promising results from splenectomy—still very small numbers.

300

The initial treatment of autoimmune hemolytic anemia (AIHA) is A. Observation and transfusion B. Corticosteroids C. Splenic embolization D. Splenectomy

Ans: B. The mainstay of treatment for both primary and secondary autoimmune hemolytic anemia’s (warm or cold) is corticosteroids. Steroid therapy is more successful in children. Splenectomy is indicated for failure to respond or inability to receive steroids for other reasons. Splenectomy works up to 80% in patients with warm-antibody AIHA.

400

Which of the following is an indication for splenectomy in a patient with thalassemia? A. >50 mL/kg year transfusion requirement B. Discomfort due to splenomegaly C. Recurrent sequestration crises D. Iron overload from transfusions

Ans: B. Thalassemias result in an inability to release oxygen and increased RBC turnover due to aberrant production of the hemoglobin chains (alpha, beta, gamma). The increased breakdown causes splenomegaly, and multiple transfusions (>200 ml/kg/yr). The iron overload may be responsible for increased infectious complications.

500

Which of the following is an indication for splenectomy in a patient with chronic myelogenous leukemia (CML)? A. Failure of chemotherapy to decrease splenomegaly B. Sequestration requiring transfusion C. Symptomatic relief of early satiety D. Presence of bcr gene mutation

Ans: C. CML (hallmark with bcr abl gene mutation) is often asymptomatic, but can also cause fatigue anorexia and LUQ symptoms including early satiety and pain (indications for a splenectomy)

Complications

100

The lifetime risk of overwhelming post-splenectomy infection (OPSI) is approximately A. 1–5% B. 5–10% C. 15–20% D. 20–25%

Ans: A. Lifetime risk is 1 – 5%. Greatest in splenectomies for hematologic disease, ages <5 or > 50.

200

The treatment of choice for splenic abscess is A. Antibiotics alone B. Percutaneous drainage C. Partial splenectomy D. Total splenectomy

Ans: D. Percutaneous and partial resections/drainage may be options for patients unable to tolerate splenectomy. Procedure of choice is splenectomy. Five distinct mechanisms of splenic abscess formation (1) hematogenous infection; (2) contiguous infection; (3) hemoglobinopathy; (4) immunosuppression, (HIV or chemo) (5) trauma. The most common origins for hematogenous spread are infective endocarditis, typhoid fever, malaria, urinary tract infections, and osteomyelitis.

300

The ideal time to give immunizations to patients undergoing elective splenectomy is A. A least 2 months before surgery B. At least 2 weeks before surgery C. In the holding area just prior to surgery D. On the day of discharge from the hospital after splenectomy

Ans: B. Vaccinations with Meningococcus, H. flu, and Pneumococcal vaccins (all encapsulated bacteria) need about 2 weeks preop.

400

Splenosis (A) usually results from failure of embryonic splenic lobules to fuse (B) results in splenetic tissue that in most cases derives its blood supply from a tributary of the splenic artery (C)results in tissue that differs histologically from the parent organ (D) often results in sufficient splenic tissue to clear intraerythrocytic inclusion bodies from the bloodstream (E) offers significant protection against OPSI

ANS: D. Splenosis is the traumatic displacement, autotransplantation, and proliferation of fragmented splenic tissue. Splenosis correlates with splenic disruption and peritoneal contamination because of poor technique, especially during morcellation and extraction, and can be avoided by careful dissection and handling of tissues. Accessory spleens result from incomplete embryologic fusion of splenic elements and have been reported to occur in as many as 15–40% of patients

500

An otherwise healthy 18-year-old male underwent splenectomy for hereditary spherocytosis. He returned a month later with complaints of diffuse abdominal pain, diarrhea, vomiting, and fever. Examination revealed a lack of peritonitis or significant distention, and amylase and lipase were within normal limits. Blood cultures were negative. A computed tomography (CT) scan of the abdomen and pelvis shows evidence of portal vein thrombosis. The following therapy should be initiated: (A) complete bowel rest with total parenteral nutrition (B) exploratory laparotomy (C) observation (D) antiulcer therapy with a proton-pump inhibitor and empiric treatment for Helicobacter pylori (E) systemic heparinization followed by long-term oral anticoagulation

Although many patients remain asymptomatic and never come to clinical attention, others present with abdominal pain, diarrhea, nausea, vomiting or fever, or several years later with variceal bleeding because of portal hypertension. Failure to institute appropriate therapy expediently can result in complications such as bowel infarction or later portal hypertension with varices and bleeding. Treatment involves systemic heparinization followed by long-term oral anticoagulation.

Randomness

100

Which of the following is NOT part of the triad seen with Felty’s syndrome? A. Rheumatoid arthritis B. Splenomegaly C. Neutropenia D. Thrombocytopenia

Ans: D. Felty syndrome = RA, splenomegaly, neutropenia. Immune complexes coat the surface of white blood cells, leading to their sequestration and clearance in the spleen with subsequent neutropenia. Neutropenia causes increased infectious risk, and drives need for splenectomy.

200

Which of the following factors does not correlate with increased morbidity in laparoscopic splenectomy? (A) splenic mass (B) blood transfusion (C) HIV status (D) hematologic malignancy (E) age

Splenectomy is often performed in HIV-positive patients for HIV-related ITP. HIV-positive status does not appear to adversely affect outcomes and splenectomy has proven effective therapy with a 93% remission rate in autoimmune thrombocytopenia.

300

The most common physical finding in a patient with hairy cell leukemia (HCL) is A. Massive splenomegaly B. Shortness of breath C. Abdominal pain D. Joint pain

Ans: A. Hairy cell leukemia patients often have minimal symptoms except massive splenomegaly. There are hair like projections seen on lymphocytes on smear. Many patients don’t require therapy. Splenectomy may return counts to normal (40-70%).

400

Which of the following is an indication for splenectomy in polycythemia vera? A. Failure of aspirin to prevent thrombotic complications B. Frequent need for phlebotomy C. Symptoms related to splenomegaly D. Prevention of progression to myeloid metaplasia

Ans: C. Pts with PV usually have a long survival usually treated by phlebotomy, ASA or chemotherapy. Occassionally splenectomy is indicated for late stage patients related to symptoms from splenomegaly (early satiety, abdominal pain, fatigue).

500

Patients with essential thrombocythemia present with A. Vasomotor symptoms B. Spontaneous mucosal bleeding C. Massive splenomegaly D. Lower extremity ulceration

Ans: A. Essential thrombocythemia can present with vasomotor symptoms, thrombohemmorrhagic events recurrent fetal loss.