Summary: Hairy cell leukemia is a rare malignant lymphoproliferative disease. It has characteristic cytochemical, morphological and ultrastructural features. We report such a case in a pregnant Malay patient. The pregnancy was terminated

Jalan Raja Muda

S K Cheong Mbbs (mal

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Med. J. Malaysia Vo!. 43 No. 1 March 1988
Hairy Cell Leukemia
A Case Report
*0 AINOON MBBS(Mal), DCP(Lond),
*R MEGAT, MBBS, DTMP(UKM),
*S K CHEONG MBBS (Mal), DCP(Lond), MRCP(UK)
**y HALIMAH MBBS(Mal), DCP(Lond)
*Department ofPathology,
Faculty of Medicine,
National University of Malaysia,
Jalan Raja Muda,
50300 Kuala Lumpur.
**Department of Pathology,
Hospital Besar Kelang,
41586 Kelang.
Summary: Hairy cell leukemia is a rare malignant lymphoproliferative disease. It has charac-
teristic cytochemical, morphological and ultrastructural features. We report such a case in a
pregnant Malay patient. The pregnancy was terminated.
Introduction
Hairy cell leukemia (HCL) is a malignant lymphoproliferative disease previously
known as leukemic reticuloendotheliosis but now well recognised as a distinct
clinicopathological entity. The disease is characterised by pancytopenia, spleno-
megaly and presence of abnormal mononuclear cells with irregular cytoplasmic
projections in the blood, marrow and other tissues, especially the spleen. It is a
rare disease (representing approximately two percent of adult leukemia) and has
not been previously reported in this country. However a case was reported in
1986 in Singapore.' The median age of onset of HCL is 50 years. Eighty percent
of the patients are male. We report here a case of HCL in a 22-year old pregnant
Malay woman.
Case Report
K.Y., a 22 year old Malay housewife, was found to have a grossly enlarged spleen
at a routine antenatal check-up at eighteen week's pregnancy. On admission,
physical examination showed marked pallor, petechial haemorrhages, enlarged
spleen which was palpable 15 cm below the left costal margin and liver which was
palpable seven cm below the right costal margin.
Full blood count was as follows:
Total white cell count: 5.5 x 10
9 /1 (N17, L80, M2 , El)
Hemoglobin: 8.6 g/dl
Red Cell count: 2.9 x 1O! 2 /1
Packed cell volume: 0.240 (ratio)
Mean cell volume: 82 fl
Mean cell hemoglobin: 29.7 pg
Mean cell hemoglobin concentration: 35.8 g/dl
Reticulocyte count: 2.6%
Platelet count: 30 x 10
9 /1.
62Peripheral blood film showed that 56% of the lymphoid cells had abnormal
morphology - large cells with abundant cytoplasm and hairy cytoplasmic
projections as shown in figure 1. The nucleus of the abnormal lymphoid cells
appeared mature and eccentric.
Bone marrow aspirate:
Hypercellular bone marrow in which 77% of nucleated cells were lymphoid cells.
The "hairy" nature of the lymphoid cells was not conspicuous except at the thin
end of the bone marrow smears. Erythropoiesis and granulopoiesis were depressed.
Megakaryocytes were normal in number and morphology.
Tartrate-resistant Acid Phosphatase Reaction:
Only 15% ofthe lymphoid cells showed positive reaction.
Surface Marker Studies:
Surface immunoglobulins were demonstrated on 85% of the lymphoid cells.
Ultrastructural Studies:
Typical hairy cells as illustrated in figure 2 were seen. No ribosome lamellar body
was observed.
Progress Note:
Her pregnancy was terminated. She was then given supportive treatment as she
refused splenectomy. She was discharged but defaulted on follow-up.
Discussion
Hairy cellleukemia was first described in 1958 by Bouroncle et a1.
2 The clinical
course and diagnostic criteria of this chronic lymphoproliferative disorder have
been further defined in many subsequent series.l :" The two main reasons why
this uncommon disease has attracted the attention of clinical investigators are
the unusual features of the hairy cells and the impressive clinical response
observed with two drugs - interferon and deoxycoformycin.
The identification of the characteristic hairy cell, with abundant cytoplasm and
irregular villous outline is one of the essential features in the diagnosis of HCL.
A diagnosis, suspected on that basis often requires confirmation by one or other
means. Bone marrow aspirate would show a lymphoproliferative process and the
hairy morphology is usually inconspicuous as is the case with our patient. In most
cases trephine biopsy demonstrates an increase in reticuline.
A unique cytochemical profile is given by positive tartarate-resistant acid phos-
phatase reaction and the characteristic crescentic arrangement of positive granules
by alpha-napthyl acetate esterase reaction not seen in any other B or T cell dis-
order. The acid phosphatase reaction is not a prerequisite for the diagnosis of
HCL. Surface marker studies demonstrate B cell characteristic in most cases. In
some, ultrastructural study by transmission electron microscopy is necessary to
ascertain the nature of the proliferating cells, where the cells show a mature
nucleus, infrequently a nucleolus and characteristic long cytoplasmic villi.
63The treatment of HCL is primarily directed to relieving the consequences ofpan-
cytopenia. Indications for therapy include massive splenomegaly, bone lesions or
a high white count [30,000 cells per microlitre}. Approximately 10 percent of
patients have an indolent course requiring no specific therapy, and these patients
are usually elderly men whose spleens are not palpable.
Splenectomy has been the mainstay of treatment as it corrects the pancytopenia
in most patients and improves their survival when the spleen is enlarged more than
five cm below the costal margin." However splenectomy has long lasting benefits
only in a minority ofpatients because the bone marrow is also affected." This and
the fact that in 10-20% of patients the spleen is not enlarged lead to about 80%
of patients requiring other forms of treatment at some stage. Chlorambucil was
formerly popular but has now teen superseded by interferon. Chlorambucil has
ameliorated the anemia and thrombocytopenia but persistent neutropenia has
resulted in a 25% mortality rate from infectious complication."
The use of alpha-interferon was first described by Quesada et al in 1984.7 Most
studies done subsequently, showed a return of blood count to normal in 70 to 100
percent of patients with complete eradication of hairy cells in the peripheral
blood and the bone marrow in a small minority. Therefore although interferon
is highly effective it is rarely curative. Spiers et al have recently reported good
result of treatment with the adenosine deaminase inhibitor 2-deoxycoformycin.s
Studies done have shown that in contrast with interferon, the duration of treat-
ment required to obtain complete bone marrow remission is relatively short and
prolonged remissions without maintenance treatment have been recorded in some
patients.
Thus in the past five years two highly effective drugs have been developed
for this rare disease. In deciding how to employ this new treatment as well as the
former standard treatment, splenectomy, Golomb et al has come up with a recom-
mendation:? Patients with adequate blood counts and a non-palpable spleen
need not be treated at all. Splenectomy is recommended for patients with cyto-
penias and a large spleen and they may not need any further therapy. In contrast,
patients with a diffusely involved marrow and a spleen that is not palpable usually
need further therapy within six to 12 months. Patients who fall between these
two extremes present a more difficult problem, and their therapy must be indivi-
dualized.
References
1Tan CC, Suri R: Hairy cellleukemia - A rare lynphoproliferative disorder. Singapore Med. J. 198728:2:
192-195.
2Bouroncle BA, Wiseman BK, Doan CA: Leukemic reticuloendotheliosis. Blood 1958; 13 : 609-30.
3Catovsky D. Hairy cellleukemia and prolymphocytic leukemia. Clinical Haematology 1977; 6 : 245-68.
4 Colomb HM, Catovsky D, Golde DW: Hairy cellleukemia; a clinical review based on 71 cases. Ann Intern
Med. 1978; 89: 677-83.
SJansen J, Hermas J, for the Collaborative study group. Splenectomy in hairy cellleukemia. A retrospective
multicenter analysis. Cancer 1981; 47:2066-76.
6Golomb HM, Schmidt K, Vardiman JW. Chlombucil therapy of twenty-four postpslenectorny patients with
progressive hairy cellleukemia. Semin. Onco11984; 11: Suppl 2:502-6.
7Quesada JR, Reuben J, Manning JT, Hersh EM, Gutterman JV. Alpha interferon for the induction remission
in hairy cellleukemia. N Engl J Med 1984; 310: 15-8.
sSpiers ASD, Parekh SJ, Bishop MB. Hairy cellleukernia: induction of complete remission with pentosta-
tin (2-deoxycoformycin). J Clin Onco11984; 2: 133-42.
9Golomb HM, Ratain MJ. Recent advances in the treatment of hairy cellleukemia. N Engl Med 1987; 316
870-71.
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