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Malignant Histiocytosis of the Robb-Smith Type (Histiocytic Medullary Reticulosis) Michael F

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Malignant Histiocytosis of the Robb-Smith Type (Histiocytic Medullary Reticulosis) Michael F Henry Ford Hospital Medical Journal Volume 21 | Number 1 Article 2 3-1973 Malignant Histiocytosis of the Robb-Smith Type (Histiocytic Medullary Reticulosis) Michael F. Macken Ellis J. Van Slyck Sheik M. Saeed Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Macken, Michael F.; Van Slyck, Ellis J.; and Saeed, Sheik M. (1973) "Malignant Histiocytosis of the Robb-Smith Type (Histiocytic Medullary Reticulosis)," Henry Ford Hospital Medical Journal : Vol. 21 : No. 1 , 4-10. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol21/iss1/2 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Henry Ford Hosp. Med. Journal Vol. 21, No. 1, 1973 Malignant Histiocytosis of the Robb-Smith Type (Histiocytic Medullary Reticulosis) Michael F. Macken, MD,* Ellis J. Van Slyck, MD,* and Sheikh M. Saeed, MD** THE morphologically descriptive term, "histiocytic medullary reticulosis", was proposed by Scott and Robb-Smith^ in 1939 for a clinicopathologically distinct, rapidly fatal disorder found in four patients. The disease was characterized A 60-year-old woman developed high fever, by fever, weight loss, generalized jaundice, anemia with pancytopenia and hepatosplenomegaly. She deteriorated lymphadenopathy, and in the terminal rapidly and died one month later, having stages, severe anemia, jaundice, pan­ shown no beneficial effect from high dose cytopenia and purpura. The pathologi­ corticosteroids and splenectomy. An cal examination revealed systemic pro­ antemortem diagnosis of malignant his­ tiocytosis of the Robb-Smith type was made liferation of histiocytes, some of which and duly confirmed by autopsy study. This demonstrated erythrophagocytosis. rare disorder of the reticuloendothelial system, heretofore designated "histiocytic In this paper we present another case medullary reticulosis," deserves recognition of this rare entity, with a review of the and inclusion among the histiocytic diseases and in the classification of the lymphomas. pertinent clinical and histological fea­ Its proper nosologic designation is sug­ tures. The proper place and designation gested. for this disease in the current classifica­ tion of the diseases of the reticuloen­ dothelial system is offered later in the text and in Table I. Case Report This 60-year-old housewife was well until August 9, 1969, when she was admitted to another hospital with bilateral flank pain of • Division of Clinical Hematology, Depart­ sudden onset. Examination revealed marked ment of Medicine. bilateral costovertebral angle tenderness and an enlarged spleen, which was palpable two- •'Department of Pathology. finger breadths below the left costal margin. The patient received only analgesics and all Address reprint requests to: Ellis J. Van symptoms subsided within 24 hours. Inves­ Slyck, MD, Henry Ford Hospital, 2799 W. tigation of the genitourinary tract disclosed Grand Blvd., Detroit, Ml 48202 only an incidental reduplication anomaly of Macken, Van Slyck and Saeed presence of a few shotty lymph nodes in the TABLE 1 posterior cervical, axillary, and inguinal DISORDERS OF THE MALIGNANT regions; an enlarged liver, palpable 4 cms RETICULUM CELL (HISTIOCYTE) below the right costal margin and an enlarged spleen, palpable 8 cms below the 1. Reticulum cell sarcoma group. left costal margin. A) Malignant lymphoma, undifferentiated. 1. Burkitt's lymphoma. Laboratory studies revealed the following: B) Malignant lymphoma, histiocytic hemoglobin 7.9 grams/100 ml and white (Reticulum cell sarcoma). blood count 3,400/cu mm. The differential 0) Malignant histiocytosis. count showed 54% neutrophils, 5% bands, 1. Leukemic reticuloendotheliosis occasional myelocytes and promyelocytes, (Acute monocytic leukemia). 12% monocytes, 25% lymphocytes, 1% 2. Malignant histiocytosis,Robb-Smith eosinophils, and 2% basophils. The type. reticulocyte count was 15.4% and the platelet 2. Hodgkin's disease. count was 77,000/cu mm. The direct and 3. Differentiated progressive histiocytosis. indirect Coombs' tests were negative. The A) Letterer-Siwe disease (in the bilirubin was 4.5 mgs/100 ml with 0.16 histiocytosis X group). mgs/100 direct reacting. Additional studies 4. Mycosis fungoides. gave the following results: serum LDH 2,000 A) Sezary's syndrome. units/IOOml. Prothrombin time 15 seconds with a control of 12 seconds. Partial throm­ boplastin time was 88 seconds with a control of 61 seconds, and fibrinogen was 125 both collecting systems. Because of the mgs/100 ml. Urinary hemosiderin test was unexplained splenomegaly, evaluation of the positive. The fasting blood sugar, BUN, hematopoietic system was done, yielding creatinine electrolytes and serum protein normal results for the following tests: electrophoresis were within normal limits. hemoglobin, hematocrit, bilirubin, urinary Cultures of the urine, blood, sputum and urobilinogen, Coombs'test, heterophile ag­ bone marrow were negative. Right iliac bone glutination, lupus erythematosis preparation, marrow examination showed marked nor­ serum lactic dehydrogenase, protein elec­ moblastic hyperplasia with an M:E ratio of trophoresis, alkaline phosphatase, brom­ 1:1. Occasional abnormal neoplastic histio­ sulphalein retention, blood urea nitrogen, cytes were seen, some of which showed creatinine and calcium. Radiographs of the phagocytosis of erythrocytes, normoblasts, chest and lumbosacral spine were normal. leukocytes and even platelets, (Figure 1). However, her white blood count was 3,100 with a left shift of the neutrophils. Platelets The patient's condition rapidly deterio­ were decreased. The bone marrow showed rated with deepening jaundice and increasing a decreased M:E ratio (1:1) as a result of anemia. Daily afternoon temperature spikes increased erythropoiesis. Diagnosis on dis­ reached 105°F. She was treated with predni­ charge from the hospital was "probable ure­ sone, 60 mgs per day, but showed no teral calculus and hypersplenism of undeter­ improvement. Transfusions with fresh whole mined etiology." blood were given, followed by splenectomy and biopsy of the liver and the periaortic The patient did well for the next three nodes. Following the operation*, the weeks and then developed spiking daily tem­ peripheral blood showed increasing num­ peratures, a seven-pound weight loss and a bers of neoplastic phagocytic reticulum cells feeling of apprehension. Noting icterus, (2-4%) (Figure 2), increased polychromasia, hepatomegaly and further enlargement of spherocytosis, basophilic stippling, Howell- her spleen, her physician referred her to the Jolly bodies, erythroblastosis and a pro­ Hematology Division of the Henry Ford Hos­ nounced granulocytic left shift. The patient pital. continued to do poorly and died 48 hours later. Physical examination now revealed an acutely ill, jaundiced female with blood pres­ sure of 120/80,pulse of 84/minute and temper­ ature of 101°F. Head, eye, ear, nose and throat, cardiopulmonary and genitourinary •Surgical consultation and splenectomy were examinations were within normal limits. The done by Joseph P. Elliott, Jr., MD, General significant physical abnormalities were the Surgery II. Malignant Histiocytosis of the Robb-Smith Type jf \ Figure 1 Marrow aspirate showing two malignant histiocytes. Note ingested erythrocytes and normo­ blasts. Leishman's stain approximately x2000 r.. Figure 2 Peripheral blood. Lobulated and multinucleated histiocyte with phagocytized erythrocyte. Leishman's stain approximately x2000 Macken, Van Slyck and Saeed Figure 3 Spleen. Parenchyma is replaced by neoplastic, occasionally multinucleated, reticulum cells some of which show phagocytized erythrocytes (arrows). H & E stain x390 Pathology The right and left lungs weighed 750 and 400 grams respectively. Both lungs were con­ Surgical Specimens: gested. The right lower lobe also showed The spleen was markedly enlarged, areas of consolidation. measuring 27 x 15.5 x 8 cms, and weighed 1700 grams. The consistency was moderately The heart weighed 380 grams and showed firm. Capsule was intact, smooth and dark moderate coronary arteriosclerosis with par­ gray in color. Cut surface of the spleen had tial occlusion of right coronary artery lumen. a purplish cast with punctate areas of gray- Arteriosclerosis of aorta and larger branches white nodularity. A yellow infarcted area was present. occupied the inferior peripheral part of the spleen. The liver weighed 2600 grams. The cut sur­ face was mottled with areas of red-brown The liver biopsy specimen measured 1.0 x and yellowish color. 0.5 X 0.3 cms and had a granular yellow-green color. The abdominal lymph nodes showed marked enlargement, ranging from 3.0 to 8.0 Two enlarged lymph nodes measuring up cm in size. They were located for the most to 1 cm were also submitted and were both part around the aorta below the renal replaced by yellow-gray, soft tumor. arteries and around the pancreas. These nodes had a firm, fleshy consistency with a Cross Autopsy Findings: salmon-pink color. The bone marrow was The body was markedly dehydrated and soft and pale. No nodules were seen. jaundiced. Ecchymoses were obvious over the abdominal wall. The right and left pleural Nothing remarkable was found upon spaces contained respectively 200 ml and 150
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