|Year : 1968 | Volume
| Issue : 2 | Page : 81-84
Chloroma with autopsy reports
PP Ahluwalia, RV Agrawal, MS Rawat, Asha Kher
Medical College, Nagpur, India
P P Ahluwalia
Medical College, Nagpur
|How to cite this article:|
Ahluwalia P P, Agrawal R V, Rawat M S, Kher A. Chloroma with autopsy reports. Indian J Ophthalmol 1968;16:81-4
|How to cite this URL:|
Ahluwalia P P, Agrawal R V, Rawat M S, Kher A. Chloroma with autopsy reports. Indian J Ophthalmol [serial online] 1968 [cited 2013 May 18];16:81-4. Available from: http://www.ijo.in/text.asp?1968/16/2/81/37500
Chloroma is a rare, usually myeloblastic type of leukemia. It has the same relation to myeloid leukemia as malignant lymphoma has to lymphatic leukemia. In its most characteristic form, it is associated with greenish tumours which are usually subperiosteal and are found especially in relation to the bones of the skull, particularly of the orbit and thorax. No organ is exempt except perhaps the brain and spinal medulla (BRANNAN  . Kidney, liver, muscles and spinal meninges may be affected. The lymph glands and spleen though usually enlarged, are not always so. The pigment is believed to be protoporphyrin (SCHULTZ AND SCHWARTZ,  which has also been found in lymph glands from ordinary myeloid lenkemia.
The malady appears most frequently in young children and affects males twice as frequently as females (EDGERTON)  . Leucocytic count is usually from 14,000/ c mm to 20,000/c. mm. In the early stage the total leucocyte count may be normal. At this stage peripheral blood smear may reveal the immature cells (WINTROBE  )
Its aetiology is still unknown and has got always a fatal termination. Since its original description by BURNS  upto the year 1962, 342 cases (EDGERTON,  HAMEED, TULSIDAS AND AGARWAL  ; and MORTADA  ), have been reported in the world literature. The present paper deals with clinical and autopsy findings in a case of chloroma, in a young hindu male boy with bilateral proptosis as the main presenting symptom.
| Case report|| |
A Hindu Male child, 3½ years of age was admitted with the complaints of gradual increase in the prominence of both eyes and ulceration of the left eye of 15 days and 8 days duration respectively. There was a past history of purulent discharge from both the ears.
On examination, the child was fully conscious, pale and afebrile. Pulse - 140 per minute, respiration normal. Preauricular, cervical and submandibular lymph noes were palpable, soft and discrete. The eyelids were markedly swollen with prominent veins over its surface. The left eye had corneal ulceration [Figure - 1]. Funduscopy revealed bilateral papilloedema of less than one diopter with no other abnormality. Rest of the systems were normal.
Hoemoglobin was 50 per cent, total leucocytic count - 11,000/c mm. The leucocytic count differentiated polymorphs 80 per cent and lymphocytes 20 per cent. X-ray of the orbits (antero-posterior and lateral views) and screening of the chest revealed nothing abnormal. Sickling and Mantoux tests were negative. Conjunctival smear was negative for pathogenic organisms.
The patient was given antibiotics, hypertonic glucose and local treatment for corneal ulcer. On the tenth day of admission, he was found to be more pale and optic atrophy was noticed to be commencing. On the 17th day, he had bleeding from the left ear. General condition was poor with marked emaciation. He was conscious, febrile and had no neck rigidity. Proptosis had increased and urticarial rash was seen all over the body. Spleen was 1 cm. palpable. Total leucocytic count was 20,000/c mm and in the peripheral smear plenty of immature blast series of cells were seen.
The patient continued in the same unsatisfactory condition for three weeks. On the 42nd day of his admission he became apnoeic after taking food and expired immediately, inspite of attempts at resuscitation.
The body was that of a young, markedly pale and emaciated male child. The preauricular, submandibular, cervical, axillary, inguinal, bronchial and mesenteric lymph nodes were enlarged; they measured 1 to 2 cm. in diameter and were soft and discrete. On sectioning, they showed a greenish colour. Besides the lymph nodes, green colour tumour masses were seen in the liver and the kidneys [Figure - 2]. In the liver, the tumour was localised in the portal tracts. The spleen and lungs revealed greenish colouration, but no visible tumour mass.
The eyelids and paraorbital tissue on both sides had firm, green, irregular tumour masses. The eye balls were normal. In central nervous system, superior sagittal and both the lateral sinuses were occupied by a firm, homogenous thrombus [Figure - 3]. Green pus was aspirated from the right middle ear. Bacteriological culture from the pus, thrombus and blood was positive for pneumococci.
Microscopically, the tumour in every organ was composed of cells with a prominent pale nucleus with nucleoli and indistinct cytoplasmic borders [Figure - 4],[Figure - 5]. These cells were seen infiltrating the superior sagittal and the lateral sinus wall with tumour thrombus in the lumen. The intestines and both the eyeballs showed microscopic infiltration. [Figure - 6].
| Discussion|| |
Proptosis is the most common presenting symptom in cases of chloroma (MALKIN,  GOOD MAN AND IVERSON,  and DUKE-ELDER  . If the patient lives long enough, corneal damage, immobility of globe, papilloedema, retinal haemorhage, optic atrophy and blindness may result (DUKE-ELDER  ). The present case had bilateral proptosis as the presenting symptom. Bilateral papilloedema was recorded at a very early stage followed by corn mencing optic atrophy.
Leucocytic count was normal in the early stage in the present case. In the later stage, immature cells were seen in the peripheral blood. The chloroma cells were infiltrating the superior sagittal and lateral sinus walls with thrombus. The thrombus was not accompanied with any neurological symptoms.
Death is usually due to disturbance of metabolism, exhaustion, cerebralmyelitis, septiczernia or pneumonia (DUKE-ELDER, 1952). Our patient died due to septicemia and marasmus.
| Summary|| |
Clinical and autopsy findings of a case of chloroma in a male child with bilateral proptosis as the main presenting symptom is reported.
In the terminal stage, the peripheral blood smear showed the presence of chloroma cells. In the post-mortem, green tumours were observed in the eyelids, both the orbits, lymph glands, liver, kidneys, lungs and spleen. Chloroma cells were also seen infiltrating the uveal tracts of the eye ball, intracranial sinus wall with tumour thrombus and the intestines. The patient died within two months of his first symptom due to septicoemia and marasmus.
| References|| |
|1.||BRANNAN, D. (1926): Bull. Johns Hosp. Hosp. 38: 189. |
|2.||BURNS, A.: (1827): Quoted by Ref. No. 3. |
|3.||DUKE-ELDER (1952): Textbook of Ophthalmology, Vol. V. Lond., Henry Kimpton. p. 5556. |
|4.||EDGERTON (1947): Trans. Amer. Ophthal. Soc. 45: 376. |
|5.||GOODMAN C. G. AND INVERSON L.: Chloroma - A clinico-pathologic Study of Two Cases. Amer. J. Med. Sci. 211, 205, (1946). |
|6.||HAMEED S., TULSI-DAS AND AGARWAL K. C.: Chloroma of the Orbit. Brit. J. Ophth. 43, 107 (1959). |
|7.||MALKIN, B. M. (1925): Quoted by Ref. No. 3. |
|8.||MORTADA, A. Orbital lymphoblastomas and acute leukemias in children. Amer. Jr. Ophthal. 55: 327, (1963). |
|9.||SCHULTZ J. AND SCHWARTZ S.: The Chemistry of Experimental Chloroma II. Isolation of Crystalline Protoporphyrin, Its Origin and Relation to Other Porphyrins. Cancer Research 16, 565, (1956). |
|10.||WINTROBE, M. M. (1961): Clinical Hoematology. Philadelphia, Lea & Febiger. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]