Immunoblastic Sarcoma
DONALD M. KURTZ, M.D.
Kurtz, Donald M.: Immunoblastic sarcoma. Am J Clin Pathol
67:227-229,1977. A systemic, malignant disorder of immunoblasts has been reported to occur as a late transition phase
of immunoblastic lymphadenopathy in an older population.5
With a background of polyclonal gammopathy, drug sensitivity, lupus erythematosus or rheumatoid arthritis, there may
develop a progressive "cloning out" of monomorphous immunoblasts to form immunoblastic sarcoma. Recently, a 17year-old, postpartum girl without these bizarre hypersensitivity phenomena developed a rapidly fatal, systemically
malignant process with severe clinical complications. No
historical incitent was identified. The pregnancy had been
uneventful and birth had produced a thriving, viable infant.
This case may represent the youngest patient with primary,
malignant immunoblastic disease occurring in the absence of
a "hypersensitivity" state. A brief discussion of the clinical
situation, management, and autopsy findings is presented.
Basic morphologic criteria for proper differentiation of malignant immunoblastic problems, lymphoma, and Hodgkin's disease are discussed. (Key words: Immunoblastic sarcoma;
Angioimmunoblastic lymphadenopathy.)
THE CASE OF a young patient with immunoblastic
sarcoma is presented.
Report of a Case
A 17-year-old white girl, gravida 1, para 0, abortus
0, was admitted to the hospital with fever, painful
left inguinal adenopathy, and cellulitis of the buttocks.
A urine culture was negative; leukocyte count was
13,000 with a slight "leftward" shift; chest x-ray
disclosed no abnormality. Treatment with broadspectrum antibiotics was begun, and the patient was
discharged. Fever continued and a hoarse, rasping,
nonproductive cough ensued. A "bronchitis" became
marked, lower-extremity edema developed, and abdominal tenderness preceded the onset of labor, which
produced a viable, unaffected infant. A reversal of the
albumin/globulin ratio (1.5/3.4 g/dl or .23 mmol/1 34 g/1)
was recorded. Results of all febrile agglutinin studies,
including toxoplasmosis, were negative. Blood cul-
Columbus Pathology, P.C.,
The Medical Center,
710 Center Street,
Columbus, Georgia 31902
tures yielded no growth. A bone marrow aspiration
showed granulocytic hyperplasia without neoplasm.
A leukemoid, leukoerythroblastotic peripheral blood
picture evolved. Lymphadenopathy progressed, and a
biopsy of a left cervical lymph node was initially
interpreted as "probable" Hodgkin's disease (Fig. I).
Steroids were provided. Blood-gas studies showed
severe acidosis with poor respiratory exchange. A
decreased platelet count and a prolonged partial thromboplastin time initiated disseminated intravascular coagulation. Repeated cardiorespiratory arrests occurred
and resuscitation was eventually unsuccessful.
Autopsy disclosed diffuse lymphadenopathy and
hepatosplenomegaly. Both lungs were consolidated,
and marked interalveolar septal hyaline membranes
were identified (Fig. 2). Examination of many lymph
nodes from all body areas disclosed focal necrosis
consistent with massive steroid therapy. In those portions still preserved, the nodal architecture was diffusely replaced by moderately enlarged, polymorphic
and pleomorphic cells (Fig. 3). Occasional abnormal
mitotic figures were found throughout the lymph
nodes. This process was identified within the spleen,
focally within the bone marrow and liver, and along
intrapulmonary alveolar septa. Respiratory insufficiency and failure were causes of death.
Received February 25, 1976; received revised manuscript May 3,
1976; accepted for publication May 3, 1976.
Address reprint requests to Dr. Kurtz.
227
Discussion
Lukes and Collins4 have described tumors of immunoblasts occurring in patients with chronic, abnormal immune disorders. These lymphomas are considered "immunoblastic sarcoma" of B cell type,
usually manifesting plasmacytoid features. Morphologically monomorphous proliferation of large pyroninophilic cytoplasmic cells with varying differenti-
KURTZ
A.J.C.I'. . Maich L977
FIG. I (left)- Left cervical lymph node initially interpreted as "probable" Hodgkin's disease.
FIG. 2 (right). Consolidated lung with marked interalveolar septal hyaline membranes.
ated plasmacytoid features is seen. The cell nucleus
may be oval with finely distributed pale-staining chromatin and two to three nucleoli. The lack of vascular
proliferation allows differentiation from angioimmunoblastic lymphadenopathy, described by Frizzera,
Moran and Rappaport. 1
The clinical laboratory was faced with the immediate
evaluation of this young patient's hematologic status
shortly following delivery. Her initial leukocyte count
of 13,000 quickly increased to more than 75,000
with the presence of nucleated erythrocytes and
markedly immature granulocytic elements. Examination of the bone marrow disclosed the leukemoid
features of this hyperplastic response. Since the
patient's clinical condition was deteriorating rapidly,
and several "shotty" cervical and axillary lymph
nodes became palpable, a biopsy with request for
frozen section interpretation was performed. Atypical
lymphoreticular hyperplasia and "probable" Hodgkin's disease were subsequently reported.
Because of the severe clinical situation and the
opinion that this was a malignant, systemic process,
consultation was requested for confirmation of a dif-
ficult morphologic problem and the efficacy of using
systemic chemotherapeutic agents.
Enzyme histochemical studies performed by the
Armed Forces Institute of Pathology suggested that the
lesion was not granulocytic leukemia or a neoplasm
of histocytic or monocytic origin.
Dr. Rappaport's evaluation was concerned with the
proliferation of lymphoid cells of various sizes, which
with the methyl green-pyronine stain showed intense
pyroninophilia.
Drs. Lukes and Tindle, in their review of the case,
classified the changes seen as malignant lymphoma,
immunoblastic sarcoma, on the basis of the large
transformed lymphocytes with the abundance of plasmacytoid cells, seen in the initial lymph-node biopsy
specimen.
1 believe this case may represent the youngest
patient with initial malignant immunoblastic disease
thus far reported. The patient had no past history of
any serious individual or family health problem.
There appears to have been no hypersensitivity background in this case to account for the development of
immunoblastic disease. The pregnancy was unevent-
IMMUNOBLAS'TIC SARCOMA
vol. 67. No. 3
229
ful, and there was no indication of a chronic immune
disorder.
Critical reviews of the morphologic histologic classification of lymphomas and Hodgkin's disease by
several investigators have shown the difficulty with
initial examination. Careful evaluation of microscopic
morphology points out three cardinal features: (1) the
absence of characteristic Reed-Sternberg cells; (2) the
plasmacytoid features of transformed lymphocytes;
(3) the lack of vascular proliferation within the lymph
node.
This observer and others have pointed out previously
the difficulties of proper lymph-node evaluation under
variable conditions. 2 ' 3,6 Using established histologic
technics with proper fixation, sectioning and staining,
an exhaustive search for acceptable Reed-Sternberg
cells should be made in every case of atypical lymphoreticular proliferations.
Despite massive steroid therapy, disseminated intravascular coagulation with formation of acquired hyaline membranes introduced irreversible pulmonary insufficiency. Careful evaluation of the pulmonary interalveolar septa suggested the presence of immunoblastic sarcoma cells along these distorted membranes.
The possibility that this was the site of action of an
abnormal "immune" response with formation of the
acquired hyaline membranes is an interesting speculation.
Awareness that this disease is not confined to the
elderly should place the differential diagnosis of
Hodgkin's disease, lymphoma and reactive nodal
proliferations in a more proper perspective.
Acknowledgments. Drs. J. Crowder and D. Cabaniss allowed the
author to study their patient. The Aimed Forces Institute of
Pathology, Drs. H. Rappaport, R. J. Lukes, and B. Tindle
consulted in establishing the diagnosis.
References
1. Frizzera G, Moran EM, Rappaport H: Angioimmunoblastic
lymphadenopathy with dysproteinemia. Lancet, June 1, 1974,
pp 1070-1073
2. Kurtz DM: To biopsy or not to biopsy. JAMA 214: 1888.
1970
3. Kurtz DM: To biopsy . . . revisited. JAMA 216:522.
1971
4. Lukes RJ, Collins RD: Immunologic characterization of human
malignant lymphomas. Cancer 34:1488-1503, 1974
5. Lukes RJ, Tindle BH: Immunoblastic lymphadenopathy—A
hyperimmune entity resembling Hodgkin's disease. N Engl
J Med 292:1-8, 1975
6. Saltzstein SL: Value of biopsy in Hodgkin's disease. JAMA 215:
984, 1971