Monocytoid B-cell lymphoma with a distinctive clinical presentation

HUMAN PATHOLOGY

MONOCYTOID Motf;\blhl,\t)

VASH’.

B-CELL

LYMPHOMA

MD. ANI) ~‘1I.l.l.w

WITH

Volume 24, No. 5 (May 1993)

A DISTINCTIVE

CLINICAL

PRESENTATION

k:. t*LYf%IN, 1.~11). PHI)

wntation.” This case report MBCI. ilt which the paCcnc ;I mild Ivniptio~~losi~. (:ASt:

Monocytoid B-cell lyntphoma (MBCI.) is an unusual form of non-Hodgkin’s K-cell lyntphoma that was originally described by Sheibani et al in 1986.’ This B-cell lyntphonta is thought to be the neoplastic counterpart of tttottocytoid B lyntpltocytes. Monocytoid B cells’ or “immature sinus histiocytes,“” were originally described in toxoplastttic Iyntphadenitis. They also are seen in ;I variety of orhet- react&e conditions.“-” These cells have regular round to oval nuclei and abundant clear cymplasni, and express B lymphocyte-associated antigetts.‘,“.7.” Since its original descriplion there have been many other reports indicating that MBCI, is typically a disease of peripheral lymph nodes.“~” Peripheral blood and bone marrow involvenlent is rare in this type of lymphottta.“~‘” Splenic involvement is rare and the few reported cases also had peripheral lytnph node involvemenr at the time of l-“-e-

describes presw(ed

an unusual exatttl~lr of with sl~lenontega1y ant1

KEPOKI‘

An Wyear-oIt1 white ttim prescm~c’d itt hlay I!)!)0 with slowly increasing abdominal girth. epigtstCcpaitt, :tttd earl> satiety. .4n ~tbdortiinal c.otnpulcd rottiogr~tph~ s(~att rcvc~ilcd tttarked splettotttegaly and rc~roperitoneal lylttl)h”dettol’atll). Hetnatologic evaluariott r-evealed ;I white blood cell court1 of X.5 X 10” cells/l, (normal rartgr. 4.5 to I I X IO” cells/l.) with 135% xepir~itccl netttrophils, 47% Ipriipltocyccs;. and 10% tttonoc‘ytes. There wab ;I slight normocytic. norttto~hrontic memia (hemoglobin, I I2 g/L; ttormal range, 120 10 I50 g/l.) a11dmoderate throntbo~ytol,eliill (93 X IO” platelets/L; normal range. I30 to 110 X IO! platelets/L). Serum clccrrophoresis showed no evidence of ;I ntonoclottal proicin. No peripheral lyttipliadenopath); was noted. A bone mat-row biopsy rrvealed 01tly: occasional nonl~;tr;ttrat~e~ular, cytologic-ally benign lyntphotd aggregares. .l‘he results of’ immunophetio~yl~ing of peIripheral blood I~tttphocyIe5 by flow cytontett) were interpreted as compatible wth &&t-chronic lytttphocytic Ieukentia ((:LI,) or a leukentic phase of non-Hodgkin’s lymphonta. Followittg ;I course of spletiic- irradiation the patient uriderwctir splencctonty and biopsy of an enlarged retroperitoneal I)ntplt node. Based in part on the patient’s age. no furthettl-eaLtttt’ttt wab provided. The pa’iettL died in December 199 I (20 ntott~hs after the initial presentation) with estettsivr pet-ipheral Iytnptt;idenop;trhy. Cervical. axillary. and inguinal lymph 1tc&3 were all enlarged. An ;tutopsy was not pcrfornted. MATERIALS

AND

METHODS

Portions of spleett and Iyntph ~tocle were fixed in 135, 10% buffered formalin, atnd Hollandes fixatives. pt.ocSessed I-ow tittely, and stained with hentaroxylili-eositt for light mic.roscopic examination. Additional fresh tissue was frozen for inttttunohistology. A portion or the lymph node wa:, used (0 prepare ;I cell suspension for flow cytometry and itttttturto~ytolo~~. Fresh tissue WIS mechanically dissociated using ;I (iO-pm coppet mesh. The cells were collected and washed in Hank’s balancecl

558

CASE

TABLE

Il. Primary Antibodies Sections and on Cytospin

Used on Cryostal Preparation

2x11solutioli. .I‘hr c,rll suspension was Ilien fittti-etl a dnglc cell suspension. p111 Nitcxx ti11~1. 10 cdhll I;rc)/vil sc.c’iic~nirri~nuiiot~isloto~~ and +ospiii

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FIGURE 2. Retroperitoneal lymph node showing a residual lymphoid follicle (arrow) and infiltration of neoplostic cells in an interfollicular and sinusoidal oattern.

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SpltWl. .I‘hr. spleen showed ;I t)i-~~loriiinaritl~ iiotlul;ii- iiifittralr 01 I\riiptioid ~~11s inorphotogic-;ill~ itl~iiiic al lo ~tiosr sccii in ttw rr(I‘opcri1oIieat lymph ncdv. In IJi;iny areas ttit. n~oplastic cells had ;I distinctivr niaixjn;il /on<’ distribution (Fig 3) with presrrvatic 01 of the noi-nial periar-lri-ial Iynqhocc~~ cufY. In v)nic‘ ;irc;is the atypical crlts infillratrtl rtic spleriic cords, crratiiig ;I diffuse pa”wii with (oalc~~ rii(‘t‘ of dclj;icerii notlules. No “l~lootl hkes” were prc’ww~,

Immunologic

Observations

ININt1~)/0hi.\t0[0g~. Fromii se< tical iririr~ui~~c~tiisloio~~ wax performed 011 tissue from the reti.op~ritori~~~l Ivnlph node using

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of small Ivmphocyte~ showed (:1)5 and (:I13 im~~~unoreacrivi~~. These ccl14 nlost likely rep‘I‘ Ivinptic~cvl~~. Definite CD.5 rcscnl wactivr or rcsiduat normal iliiiiiiiriol.r;ic-kibitz of rhe large cells was Iiot iiol~~t. ImmunoIii atlditic~ri.

FIGURE 1. Peripheral blood smear showing a large lymphoid cell with slightly indented nucleus and abundant cytoplasm with irregular cytoplasmic projections.

559

;I significanl

rractivc~

Iiuiriber

HUMAN PATHOLOGY

Volume 24, No. 5 (May 1993)

includes well-differentiated lymphocytic lymphoma/CI.I. and hairy cell leukemia (HCL). The patient presented with massive splenomegaly and mild peripheral blood lymphocytosis without peripheral lymphadenopathy. This distribution of disease is more typical of leukemia than lymphoma and hy itself is suggestive of HCL. The neoplastic cells in this case did not express CD25 and had only weak cytoplasmic arid phosphatase that was sensitive to tartrate. Hairy cell leukemia and MBCL do share many morphologic and phenotypic properties; furthermore, they may both show a sinusoidal and/or interfollicular pattern in lymph nodes.“,” The neoplastic cells of MBCL and HCL are almost indistinguishable based on morphologic features alone.“,“,“’ Phenotypically both MBCL and HCL coexpress CD1 Ic (Leu-M5) and B-cell lineage-specific antigens, 1.14.X1.% Genotypically. several studies have shown clonal rearrangements of immunoglohulin heavy chain.‘,“,“,“’ Hmson et al recently reported 13 cases of a chronic lymphoproliferative disorder that also is related to both (:I,I. and HCI, and that also shares some of the features of our case.L”’ Eleven of the 14 patients reported by Hanson et al had splenomegaly that was “massive” in five cases. CD5 was expressed in half of those cases and expression of CD2.5 was negligible in all hut two cases. Rarely, MBCI, cells may express CD5.” hy cirIn our case tlow cytometry revealed CD5 expression culating neoplastic cells and cells from the retroperitoneal lymph node. CD5 immunoreactivity was not detected in frozen sections of the retroperitoneal lymph node. The reason for this discrepancy is uncertain; this might be due to differences in the sensitivities of the two methods. In the cases reported by Hanson et a1, only two patients had peripheral lymphadenopathy. However. in contrast to our case, the leukemic cells reported by Hanson et al were morphologically similar to those of CLI.. Furthermore. leukocytosis was a striking feature in the white blood cell most of their patients. In one patient

FIGURE 3. Spleen showing a distinctive marginal zone distribution of MBCL cells. The periarterial lymphocyte cuff is partially preserved.

cytologic stains using cytospin preparations from the retroperitoneal lymph node demonstrated irnmunorea~tivity fol HLA-DR in the neoplastic cells. Staining for Cl)25 (II.-2 receptor/TAC) was negative. Flour Cytor~tq. Two-color Ilow cytometry was performed on circula&g neoplastic cells from peripheral blood and on a cell suspension obtained from the retroperitoneal lymph node using a pane1 of phenotypic markers (Table 2). The neoplastic cells showed immunoreactivity with CD 10 (Leu-12). CD20 (Leu-16), CD21. CD22 (Leu-14). CDI 1~.(l,e~M5). and CD5 (Leu-I). In the peripheral blood approximately 34Yo of the lymphoid cells co-expressed CD 22 and CD1 lc, and approximately 51% of the lymphoid cells co-expressed CD1 9 and CD5. In the lymph node-derived cell suspension nearly all of the larger lymphoid cells co-expressed CD 1 1c and CD22 and they expressed only kappa light chain. Staining for CD4, CDS, CD16. ancl CD10 (CAI.1.A) was negative.

count

was 131 X IO” cells/I.. Another lymphoproliferative clisorcler that alao may be related to MBCL is splenic lymphoma with villous lymphocytes. l’atirnts with this disorder typically have circulating neoplastic cells with fairly abundant cytoplasm and thin cytoplasrnic projecrions.“‘,” The neoplastic cells express B-cell antigens, such as CD 19. <:D20. CD22, and CD24, hut they typically lack CDS. CD1 Ic, and CD’):i.“‘,” The distribution of malignant cells in the spleen of WI

DlSCUSSION In 1986, Sheibani et al reported several unusual cases of non-Hodgkin’s lymphoma containing a neoplastic population with morphologic features similar to monocytoid B cells; these cases were designated as MBCL.’ Since then many similar cases have been reported.“~‘““~“‘~“’ Most patients with MBCI, are elderly and peripheral lylilphadenopathy is the most COIIIIIIOII clinical presentation. A minority of patients ~nay present with extranodal MBCL in organs such as the parotid gland, hreast, stomach. nasopharynx, tonsils, and thyroid.!‘.” The majority of the reported cases with extranodal MBCl. also have prripherdl lymph node disease at the time of presentation. Bone marrow involvement occasionally occurs in patients with MBCI,. In the series of 21 patients reported hy Sheihani et al!’ two of nine patients who had bone marrow biopsy showed marrow involvement by MBCI.. In Cogliatti et al’s study”’ onl!, three of 21 patients had documented marrow involvement lq MBCL. A leukemic phase of MBCI, is rare and all reports of such cases also had peripheral lymph node involvement at the time of presentation.“‘“’ Only three of 121 patients listed in the MBCL registry at the City of Hope National Medical Center had leukemic conversion.” All three patients presented with significant generalized lymphadenopathy. The case reported here represents a clinicall) unique example of MBCL. The featur-es of this case pl-ovide additional support for the proposal that MBCL should he considered in the spectrum of B-cell lymphoproliferative disordrrs that also

patient, a marginal Tone pattern, is similar to the location of nonneoplastic monocytoid B cells in that or#an.'"~"" Whether this patient’s lymphoma originated in the spleen or neoplastic

TABLE 2. Two-Color Flow Cytometric Analysis of Circulating Neoplastic Cells and Large Cells From the Retroperitoneal Lymph Node

Alltihotlie\ (:I)l!l+/(:l).‘,<:I) 1’)+/(:1)5+

c:lE+/c:n

I I( -

<:1,ss+/c:n1 IV+ c:1>1o+ (:IX(/HI.A DR+ (:lx?o+ K‘q,p;‘+ 1.amhtl;i+

560

I’elxerrragr of Positive Ixgr Cells in KecI~opel~itcJrlral Lymph Nodes 30

55 Ii X0 N I) ND ND x7 0.0

CASE STUDIES

561