632
Proc. roy. Soc. Med. Volume 70 September 1977
Clinical Section President Norman Whittaker MD
Meeting 8 October 1976
Cases Histiocytic Meduliary Reticulosis Presenting as Rheumatoid Arthritis Julie Crow, BSC MB BS and J M Gumpel, MRCP (Northwick Park Hospital, Watford Road, Harrow, Middlesex, HA] 31UJ) Mr C S, aged 52 History: Admitted to hospital with a symmetrical polyarthritis which had started with pains in the knees and ankles 4 years previously and progressed to involve the hands and wrists over the previous 6 months. He had also lost 10 lb in weight in 4 months and had experienced attacks of acute dyspnea with frothy white sputum. He had a past history of malnutrition and of tropical ulcers developing on pre-existing ichthyosis when in the Far East in World War II. He had also had a cholecystectomy. On examination: He had ichthyotic skin, generalized muscle wasting and moderate finger clubbing. Synovial thickening and effusions were present in both knees and there was limitation of extension at the wrists with dorsal tendon sheath effusions. The cardiovascular system was normal (BP 130/70) but fine basal crepitations were heard on occasion and several episodes of dyspnca were observed. The liver and spleen were both enlarged and there was moderate inguinal lymphadenopathy. Investigations: Hb 12.9 g/100 ml on admission, falling to 11.0 g/100 ml before death. WBC 7.3 x 1IO/gI on admission falling to 3.6 x I03/ Vl. Differential count normal. Reticulocytes 1 %. Platelets varied between 88 000 and 92 000 x 103/pd. ESR 50 mm/hr (Westergren) on admission, falling to 20 mm/hr before death. Prothrombin and clotting times normal. Coombs test negative. Serum B12 and folate normal. Biochemistry: all values repeatedly normal apart from minimally low serum calcium (2.10 mmol/1). Rheumatoid factor and ANF: negative. Increased serum IgA
level. ECG and chest X-ray: normal. X-rays of joints: normal. Sternal marrow: increased cellularity; normal iron stores; possibly early megaloblastic changes. Barium meal and sigmoidoscopy: normal. Jejunal biopsy, liver biopsy, rectal biopsy: no diagnostic abnormalities. Xylose absorption reduced. Facal fats: normal. Synovial biopsy (knee): a fragment of collagenous connective tissue only. Clinical course: Over the next month in the ward his polyarthritis improved but his general condition deteriorated. Weight loss and attacks of dyspnoea persisted and he became increasingly anaemic and leucopenic. During a weekend at home he collapsed and was readmitted in a shocked, hypotensive state. Within minutes he had a cardiac arrest and despite full resuscitatory procedures including an internal pacemaker, no response was obtained. Post-mortem examination: The heart showed marked right ventricular hypertrophy and there was severe pulmonary cedema. The liver (1980 g) and the spleen (1100 g) were enlarged and there was a large gastric ulcer. -Generalized lymphadenopathy was present, the abdominal nodes being the most severely affected. The right hip appeared normal and the right knee showed only slight synovial hyperplasia and congestion. Histology: Lymph nodes: the normal follicular architecture was replaced by sheets of phagocytic histiocytes with marked erythrophagocytosis around foci of necrosis (Figs 1, 2). Spleen: the red pulp was infiltrated by similar histiocytes. Liver: the sinusoids were engorged by histiocytes which had not been present in the liver biopsy taken 2 weeks previously. Histiocytic cell infiltration was also found in the interstitial tissue of the heart and lungs and to a lesser degree in several other tissues. Synovium: there was an increased vascularity and patchy loss of surface cells with deposition of fibrin. The slight cellular infiltrate which also was present was composed of both lymphoid and
633
Clinical Section Cliica Secio
Fig 1 Lymph node showing complete destruction of normal architecture (x 15.50)
mvr i-,Z_
U~~~~~~
Fig 2 Lymph node showing histiocytes and erythrophagocytosis (arrowed) ( x 1200)
633
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R
_
Proc. roy. Soc. Med. Volume 70 September 1977
634
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_
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ww*
that it resembled the nonspecific arthritis sometimes seen with malignant neoplasms, and which clinically often antedates manifestations of the a neoplasm itself (Mackenzie & Scherbel, 1963). The histiocytic infiltration of the myocardium is of interest in view of the failure of response to cardioresuscitatory procedures following his cardiac arrest. Acknowledgments: We are grateful to Dr D S Smith, under whose care this patient was admitted, and Dr G Slavin for his encouragement and help.
(('
REFERENCES Fig 3 Synovium showing increased vascularity and loss of surface cells with deposition offibrin (F) and slight infiltration with lymphoid cells and histiocytes ( x 120)
histiocytic cells (Fig 3). Stomach: of a simple peptic ulcer. Skin: ichthyosis were present.
the features
Clime M J & Golde D W (1973) American Journal of Medicine 55, 49 Mackenzie A H & Scherbel A L (1963) Geriatrics 18, 745 Scott R B & Robb-Smith A H T (1939) Lancet, ii, 194
were
the features
of
Discussion
The following cases were also presented:
systematized histiocytosis with erythrophagocytosis is characteristic of the condition described as 'histiocytic medullary reticulosis' by Scott & Robb-Smith in 1939 and considered to be a malignant neoplasm of the histiocytic (macrophage) cell series (Cline & Golde 1973). The very unusual feature in this case was the initial presentation with a polyarthritis which antedated the generalized
Hodgkin's Disease with Spinal and Brainstem Metastases Dr Victor W Johnson, Mr D J C Graff and Dr M V Salmon (New Cross Hospital, Wolverhampton, WVIO OQP)
This
illness. In this respect this disease must be differentiated
from
multicentric
derm'atoarthritis),
(lipoid and joints though, in
a
reticulohistiocytosis
condition in which skin
characteristically affected. Ala slight histiocytic infiltration was present in the synovium at postmortem examination, it is unlikely that the arwas due to these cells but rather
thropathy
are
the present case,
directly
Multiple Salpingorectal and Salpingosigmoid Fistula with other Fistult into the Bladder and onto the Lower Abdominal Wall following Surgery Mr Brian J Stoodley (for Mr J 0 Robinson and Mr C N Hudson) (Eastbourne District General Hospital, Eastbourne, East Sussex)
Proc. roy. Soc. Med. Volume 70 September 1977
Clinical Section President Norman Whittaker MD
Meeting 8 October 1976
Cases Histiocytic Meduliary Reticulosis Presenting as Rheumatoid Arthritis Julie Crow, BSC MB BS and J M Gumpel, MRCP (Northwick Park Hospital, Watford Road, Harrow, Middlesex, HA] 31UJ) Mr C S, aged 52 History: Admitted to hospital with a symmetrical polyarthritis which had started with pains in the knees and ankles 4 years previously and progressed to involve the hands and wrists over the previous 6 months. He had also lost 10 lb in weight in 4 months and had experienced attacks of acute dyspnea with frothy white sputum. He had a past history of malnutrition and of tropical ulcers developing on pre-existing ichthyosis when in the Far East in World War II. He had also had a cholecystectomy. On examination: He had ichthyotic skin, generalized muscle wasting and moderate finger clubbing. Synovial thickening and effusions were present in both knees and there was limitation of extension at the wrists with dorsal tendon sheath effusions. The cardiovascular system was normal (BP 130/70) but fine basal crepitations were heard on occasion and several episodes of dyspnca were observed. The liver and spleen were both enlarged and there was moderate inguinal lymphadenopathy. Investigations: Hb 12.9 g/100 ml on admission, falling to 11.0 g/100 ml before death. WBC 7.3 x 1IO/gI on admission falling to 3.6 x I03/ Vl. Differential count normal. Reticulocytes 1 %. Platelets varied between 88 000 and 92 000 x 103/pd. ESR 50 mm/hr (Westergren) on admission, falling to 20 mm/hr before death. Prothrombin and clotting times normal. Coombs test negative. Serum B12 and folate normal. Biochemistry: all values repeatedly normal apart from minimally low serum calcium (2.10 mmol/1). Rheumatoid factor and ANF: negative. Increased serum IgA
level. ECG and chest X-ray: normal. X-rays of joints: normal. Sternal marrow: increased cellularity; normal iron stores; possibly early megaloblastic changes. Barium meal and sigmoidoscopy: normal. Jejunal biopsy, liver biopsy, rectal biopsy: no diagnostic abnormalities. Xylose absorption reduced. Facal fats: normal. Synovial biopsy (knee): a fragment of collagenous connective tissue only. Clinical course: Over the next month in the ward his polyarthritis improved but his general condition deteriorated. Weight loss and attacks of dyspnoea persisted and he became increasingly anaemic and leucopenic. During a weekend at home he collapsed and was readmitted in a shocked, hypotensive state. Within minutes he had a cardiac arrest and despite full resuscitatory procedures including an internal pacemaker, no response was obtained. Post-mortem examination: The heart showed marked right ventricular hypertrophy and there was severe pulmonary cedema. The liver (1980 g) and the spleen (1100 g) were enlarged and there was a large gastric ulcer. -Generalized lymphadenopathy was present, the abdominal nodes being the most severely affected. The right hip appeared normal and the right knee showed only slight synovial hyperplasia and congestion. Histology: Lymph nodes: the normal follicular architecture was replaced by sheets of phagocytic histiocytes with marked erythrophagocytosis around foci of necrosis (Figs 1, 2). Spleen: the red pulp was infiltrated by similar histiocytes. Liver: the sinusoids were engorged by histiocytes which had not been present in the liver biopsy taken 2 weeks previously. Histiocytic cell infiltration was also found in the interstitial tissue of the heart and lungs and to a lesser degree in several other tissues. Synovium: there was an increased vascularity and patchy loss of surface cells with deposition of fibrin. The slight cellular infiltrate which also was present was composed of both lymphoid and
633
Clinical Section Cliica Secio
Fig 1 Lymph node showing complete destruction of normal architecture (x 15.50)
mvr i-,Z_
U~~~~~~
Fig 2 Lymph node showing histiocytes and erythrophagocytosis (arrowed) ( x 1200)
633
'
.*
R
_
Proc. roy. Soc. Med. Volume 70 September 1977
634
*
_
d
ww*
that it resembled the nonspecific arthritis sometimes seen with malignant neoplasms, and which clinically often antedates manifestations of the a neoplasm itself (Mackenzie & Scherbel, 1963). The histiocytic infiltration of the myocardium is of interest in view of the failure of response to cardioresuscitatory procedures following his cardiac arrest. Acknowledgments: We are grateful to Dr D S Smith, under whose care this patient was admitted, and Dr G Slavin for his encouragement and help.
(('
REFERENCES Fig 3 Synovium showing increased vascularity and loss of surface cells with deposition offibrin (F) and slight infiltration with lymphoid cells and histiocytes ( x 120)
histiocytic cells (Fig 3). Stomach: of a simple peptic ulcer. Skin: ichthyosis were present.
the features
Clime M J & Golde D W (1973) American Journal of Medicine 55, 49 Mackenzie A H & Scherbel A L (1963) Geriatrics 18, 745 Scott R B & Robb-Smith A H T (1939) Lancet, ii, 194
were
the features
of
Discussion
The following cases were also presented:
systematized histiocytosis with erythrophagocytosis is characteristic of the condition described as 'histiocytic medullary reticulosis' by Scott & Robb-Smith in 1939 and considered to be a malignant neoplasm of the histiocytic (macrophage) cell series (Cline & Golde 1973). The very unusual feature in this case was the initial presentation with a polyarthritis which antedated the generalized
Hodgkin's Disease with Spinal and Brainstem Metastases Dr Victor W Johnson, Mr D J C Graff and Dr M V Salmon (New Cross Hospital, Wolverhampton, WVIO OQP)
This
illness. In this respect this disease must be differentiated
from
multicentric
derm'atoarthritis),
(lipoid and joints though, in
a
reticulohistiocytosis
condition in which skin
characteristically affected. Ala slight histiocytic infiltration was present in the synovium at postmortem examination, it is unlikely that the arwas due to these cells but rather
thropathy
are
the present case,
directly
Multiple Salpingorectal and Salpingosigmoid Fistula with other Fistult into the Bladder and onto the Lower Abdominal Wall following Surgery Mr Brian J Stoodley (for Mr J 0 Robinson and Mr C N Hudson) (Eastbourne District General Hospital, Eastbourne, East Sussex)
