Surgical aspects of certain blood dyscrasias

SURGICAL ASPECTS OF CERTAIN BLOOD DYSCRASIAS DAMONB.

PFEIFFER,

M.D.

AND

Chief of Pfeiffer Surgical Clinic, Abington MemoriaI Hospital ABINGTON,

T

F.

M.

SIMMONS

PATTERSON,

M.D.

Member of Pfeiffer Surgical Clinic, Abington Memorial Hospital PENNSYLVANIA

are certain bIood dyscrasias arisen that this perversion may have as a that respond miraculously to surgery. part basis nutritiona fauIts such as There are others for which surgery vitamin B and C deficiencies. gives pahiative relief, and there are still Familial Hemolytic Anemia. This is others for which surgery offers nothing. the first blood dyscrasia that we wish to Surgery in these bIood dyscrasias is mention that responds nobIy to spIeneclimited mainIy to spIenectomy. The spIeen tomy. This condition is a chronic famiIia1 has been caIIed an organ of mystery, but and congenita1 anemia characterized by it is not essentia1 to Iife, because following jaundice, spIenomegaIy, increased fragiIity its surgical remova its function is adeof the erythrocytes, spherocytosis of the quateIy taken over and compensated for red ceIIs, and the occurrence of “crises.” These crises are characterized by attacks by other tissues of the body. A spIenectomy shouId never be done in the treatment of of nausea and vomiting accompanied by blood dyscrasias unless the surgeon has a pain over the spIeen, deepening of the thorough knowIedge of these diseases and jaundice, chiIIs and fever. The spIenic has adequateIy studied the case in question. enIargement, the characteristic microscopic The spleen is a compIex organ, and its picture of red bIood ceI1 engorgement in functions are many. Its physioIogy is very the spIeen, with Iarge amounts of hemocompIex. S&ice it to say that of its siderin, and the marked improvement foIIowing spIenectomy in typica cases a11 many and varied duties the foIIowing are of importance in its reIation to bIood indicate that hyperfunction of the spIeen dyscrasias: (I) In feta1 Iife the spIeen pIays a prominent part in the excessive is one of the sources of formation of bIood destruction in this disease. As a practicaIIy a11 of the bIood eIements. ruIe, spIenectomy is foIIowed by the (2) It pIays a very important part in disappearance of a11symptoms, but usuaIIy bIood destruction, since the spIeen contains the increased fragiIity and spherocytosis Iarge numbers of reticuIo-endotheIia1 ceIIs. of the red ceIIs persists. In many cases (3) It has some reguIatory action on the gaIIstones are present in the gaIIbIadder, fragility of the bIood, probabIy through the and choIecystectomy is here indicated. secretion of a hormone that in some way, During the past year the junior author generaIIy or IocaIIy, may affect hemareported three cases in one famiIy on topoiesis. (4) In some unknown fashion which spIenectomies were performed with it is concerned with the formation or exceIIent resuIts. destruction of pIateIets. (5) Being a part Idiopathic or Primary Tbrombocytopenic of the reticuIo-endotheIia1 system it is Purpura. This is the second bIood dysconcerned with disturbances of Iipoid crasia for which spIenectomy offers exmetaboIism. ceIlent rest&s. CIinicaIIy this is a disease There are much experimenta data to of earIy Iife, and it is often famiIia1 in substantiate these assumptions. In a11 nature. Its outstanding characteristics are of the bIood dyscrasias a perversion of a tendency to bIeed easiIy and excessively the reticuIo-endotheIia1 system is present into the skin and from the mucous memto some extent. The thought has recentIy branes, spIenomegaIy in a Iarge percentage HERE

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of the cases, and a marked diminution in bIood pIateIets. Examination of the bIood reveaIs in addition to a Iow pIateIet count a prolonged bleeding time, a norma coaguIation time, and absence of cIot retraction. The disease may be acute or chronic in nature, the Iatter being characterized by remissions. A wideIy accepted theory as to the etioIogy of this disease is that excessive thrombocytoIytic activity of the spIeen causes an increase in pIateIet destruction. SpIenectomy in this disease, first advocated by Kaznelson in I 916, offers briIIiant resuIts. Temporary improvement may be noted foIlowing medica treatment, but reIapses aIways occur. The optimum time for operation is during the quiescent period, and the best resuIts are obtained when spIenectomy is performed foIIowing the first bIeeding episode. Whether or not to operate in the acute form of purpura is a controversia1 point, but sometimes it is a necessity. If operation is performed in the acute phase, it shouId be done before the patient has bled to the point that he is a poor operative risk. On the other hand, spIenectomy is not indicated but is rather contraindicated in secondary thrombocytopenic purpura due to severe infections or intoxications. The treatment here Iies in satisfactoriIy attempting to contro1 the disease or factor which is causing the pIateIet destruction. Splenic Anemia. This third disorder aIso responds briIIiantIy to spIenectomy if the Iatter is done earIy in the disease. Many cases are thus permanentIy cured, and many more Iead usefu1 Iives for Iong periods of time. As OsIer stated, “splenic anemia is an intoxication of unknown nature characterized by great chronicity, progressive enIargement of the spIeen which cannot be correIated with any known cause, anemia of the secondary type with Ieukopenia, a marked tendency to hemorrhage, particuIarIy from the stomach and esophagus, and in many cases a termina1 state with cirrhosis of the Iiver and jaundice.” This entire symptom com-

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pIex is known as Banti’s disease, whereas the diagnosis “spIenic anemia” is justified when the earIy phenomena of spIenomegaIy and secondary anemia exist. Many authorities beIieve that in this disease the spIeen exerts in some way a depressing effect on bone marrow activity. That the spIeen pIays a great rbIe in this disease process is shown by the cures resulting from spIenectomy in earIy cases. At any time in the course of the disease spIenectomy is imperative. The earIier the operation, the greater the chance for cure. Operation even after cirrhosis of the Iiver has appeared offers many excelIent results. When spIenectomy is performed ligation of the coronary vein is worthy of a tria1. Even if operation is performed in earIy cases, gastrointestinal hemorrhage is a frequent postoperative occurrence. Gaucher’s disease, aIthough primarily a disease of Iipoid metaboIism, should be considered under the bIood dyscrasias because of the changes present in the periphera1 bIood and changes in the reticuIo-endotheIia1 system of which the spIeen is the Iargest reservoir. This disease is characterized by marked spIenomegaIy without ascites, moderate hepatic enIargement, Iymphadenopathy, bronzed pigmentation of the exposed skin and a famiIia1 tendency. BIood examination reveaIs a hypochromic anemia and Ieukopenia. This is a disease of earIy Iife and runs a sIowIy progressive course, but may terminate quickIy due to some intercurrent disease. Diagnosis may be estabIished by spIenic or bone marrow puncture for Iarge vacuoIated ceIIs known as Gaucher’s ceIIs, by Iymph gIand biopsy or x-ray examination of the bones. A disturbance in Iipoid metaboIism is onIy one phase of this disease, but the exact nature of this disturbance is unknown. In this disease the destructive action of the reticuIo-endotheIia1 system is exaggerated. SpIenectomy offers no hope of cure, but it is a good paIIiative measure when there exists a

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Iarge burdensome spIeen with thrombocytopenia and a hemorrhagic diathesis. The second bIood dyscrasia for which spIenectomy is an exceIIent paIIiative measure, but not a cure, is VonJaksch’s anemia. CIinicaIIy this condition is usuaIIy congenital and common to children of the Mediterranean races. It is characterized by marked spIenomegaIy, some hepatic enIargement, paIIor or jaundice of the skin, enIargement of the periphera1 Iymph and enIargement of the facial nodes, bones giving the chiId a MongoIian appearance. BIood examination reveaIs a marked increase in young nucIeated red ceIIs with a marked change in their shape and size, a moderate anemia, and a Ieucocytosis with a reIative Iymphocytosis. This dyscrasia is a disease of abnorma1 hematopoiesis and runs a chronic, progressive, fata course. SpIenectomy simpIy reIieves the child of the annoyance of a progressive spIenomegaIy. There are other bIood dyscrasias for which spIenectomy has been performed such as pernicious anemia and poIyThe miracuIous resuIts cythemia Vera. obtained in pernicious anemia by the use of Iiver have pushed spIenectomy into the background. PhenyIhydrazine and irradiation offer the greatest possibiIity of cure to the poIycythemia patient, and spIenectomy has not given the resuIts here to make it a standard method of treatment. RecentIy on the surgica1 service of the senior author at the Abington Memoria1 HospitaI there have been two patients with thrombocytopenia. These cases are brieffy reviewed because they teach a definite Iesson to the surgeon. CASE REPORTS CASE I. This patient was a fifteen year old white maIe who was admitted to the hospita1 because of repeated attacks of epistaxis. On admission he was bIeeding from the nose and gums. The RumpeI-Leed’s tourniquet test was positive, and the spIeen was not paIpabIe. BIood studies were typica of thrombocyto-

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penic purpura, no pIateIets being demonstrabIe. No intoxication or infection which might expIain the pIateIet absence was demonstabIe. The patient’s condition became very precarious, the bIeeding became more free and generaIized. A splenectomy was performed aIthough the patient was nearly moribund. The spleen was enIarged to twice the norma size. FoIIowing operation the pIateIet count rose to 50,000 on the sixth postoperative day, the bIeeding time decreased, and the hemorrhagic diathesis stopped. An interesting feature of this case was that a preoperative bone marrow biopsy showed a reduction of megakaryocytes, and that the megakaryocytes present were abnorma1. This pointed to the fact that the etioIogy of the thrombocytopenia was an impaired formation of the megakaryocytes which are the precursors of pIateIets. SpIenectomy, theoreticaIIy would be to no avail here. However, it was done as a Iife-saving procedure, and the resuIt was just that. CASE II. The second patient was a sixtythree year old white maIe admitted to the hospita1 because of hemoptysis, a generaIized rash and abdomina1 pain. It was found that he had long suffered from sinus troubIe, and in the distant past had received anti-Iuetic therapy. Examination revealed: (I) a rash on a11 extremities; (2) a maxiIIary sinusitis; (3) splenomegaIy; (4) moderate hepatomegaIy. BIood studies reveaIed: (I) a Ieucocytosis of I 9,500; (2) a pIateIet count of 57,000; (3) norma bIeeding and coaguIation times. FolIowing admission he bIed freeIy from his right ear. This man was suffering from a secohdary thrombocytopenic purpura secondary to a maxiIIary sinusitis. He responded we11to drainage of the sinuses, bIood transfusions, high caloric, high vitamin diet, and other appropriate medical therapy.

These two cases are exceIIent exampIes of the two types of thrombocytopenic purpura. In the first case spIenectomy was indicated and was done. A remarkable recovery foIIowed. SpIenectomy was not indicated, but rather contraindicated, in the second case. Operation might have caused a fataIity. Therefore, it is essentia1 to study the bIood dyscrasia in question thoroughIy, and be sure that operation is indicated before any such procedure is undertaken.

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SUMMARY

AND

Pfeiffer,

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CONCLUSIONS

The important functions of the spIeen in reIation to bIood dyscrasias are mentioned. 2. SpIenectomy offers exceIIent resuIts in famiIiaI hemoIytic anemia, primary thrombocytopenic purpura and Banti’s disease. 3. SpIenectomy offers paIIiative reIief in Gaucher’s disease and VonJaksch’s anemia. 4. Two cases of thrombocytopenic purpura are reviewed. I.

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REFERENCES

I. HANRAHAN, E. W., JR. and VINCENT, BETH. Surgery of the Spleen-Lewis Practice of Surgery. VoI. 6, chap. 15. z. PFEIFFER, D. B. Diseases of the SpIeen. Christopher’s Textbook of Surgery. Pp. 1354-1366, 1937. 3. YATER. Fundamentals of Interna Medicine. Pp. 177-518, 1938. 4. HUDOCK, E. B. and PATTERSON, F. M. S. Congenital hemolytic anemia. Clinics, I: 1021-1033, 1942. 5. KAZNELSON, P. Splenogene ThromboIytische Purpura. Wien. klin. Wcbnscbr., 29: 1451, IgI6. 6. ELLIOT, R. H. E. Diagnostic and therapeutic consideration in the management of idiopathic thrombocytopenic purpura. Bull. New York Acad. Med., 15: Ig7-zro, Ig3g.

WHATEVER may be the cause of shock, it must be insisted upon that the condition is a failure of the circulation. CIinicaIIy this is shown by a faII of bIood pressure. The fall in bIood pressure foIIows, rather than initiates, the onset of shock. It wiI1 occur onIy when the compensatory mechanism of the body which maintains the circuIation commences to break down. From “Surgery of Modern Warfare” edited by HamiIton Bailey (The WilIiams and WiIkins Company).