Histiocytic Medullary Reticulosis Complicating Chronic Lymphocytic Leukaemia: Malignant or Reactive?

Manoharan, A.; Catovsky, Daniel; Lampert, Irvin A.; Gordon‐Smith, E. C.; Galton, D. A. G.; ‐Mashadhani, Al

doi:10.1111/j.1600-0609.1981.tb01617.x

Cited by 50 publications

(4 citation statements)

References 19 publications

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“…Literature search revealed only 6 such cases [5–10]. At presentation with the HLH, our patient had no identified infective source despite extensive workup and multiple antimicrobials.…”

Section: Discussionmentioning

confidence: 95%

A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings

Lau

Toumoua

Chiruka

2012

Case Reports in Hematology

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Hemophagocytic lymphohistiocytosis (HLH) is rarely described in association with chronic lymphocytic leukemia (CLL), mostly triggered by disease progression or concurrent infection. A 68-year-old male received 4 cycles of fludarabine, cyclophosphamide, and rituximab (FCR) for CLL and achieved a complete response. Twenty-four days after the last chemotherapy, he presented with febrile neutropaenia and was diagnosed with HLH. The diagnosis was based upon persistent fever, pancytopenia, hyperferritinemia, splenomegaly, and hemophagocytosis on bone marrow aspirate. He began treatment with dexamethasone, cyclosporine, and etoposide. Fever resolved and hyperferritinemia improved but pancytopenia persisted. He died 13 days later from septic shock with positive blood cultures. A limited postmortem examination was performed and biopsies were taken from bone marrow, liver, and spleen. Biopsies demonstrated abundant hemophagocytosis by the activated macrophage as stained by CD68. There was no evidence of residual CLL as demonstrated by the lack of lymphocytes which was confirmed by the negative staining of CD79a. Chemotherapy appears to be responsible for the development of HLH in this patient. This is the second reported case of HLH developing after a rituximab-containing chemotherapy.

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“…Literature search revealed only 6 such cases [5–10]. At presentation with the HLH, our patient had no identified infective source despite extensive workup and multiple antimicrobials.…”

Section: Discussionmentioning

confidence: 95%

A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings

Lau

Toumoua

Chiruka

2012

Case Reports in Hematology

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“…HLH has been described in association with various types of hematologic malignancies, particularly T-cell lymphoma, although there were proven or suspected infectious triggers in a majority of these cases. [9][10][11][12][13][14][15][16][17] There have been few reported cases of CLL and secondary HLH, all of which were associated with proven infection or strongly suspected to have resulted from an infective trigger. 10,11,13,17 Of these cases, one was associated with EBV infection, and two developed in association with histoplasma infection.…”

Section: Discussionmentioning

confidence: 99%

“…[9][10][11][12][13][14][15][16][17] There have been few reported cases of CLL and secondary HLH, all of which were associated with proven infection or strongly suspected to have resulted from an infective trigger. 10,11,13,17 Of these cases, one was associated with EBV infection, and two developed in association with histoplasma infection. These infections were thoroughly excluded in our patient.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis in Chronic Lymphocytic Leukemia

Meki

O’Connor

Roberts

et al. 2011

JCO

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A 59-year-old woman with known stage 3 chronic lymphocytic leukemia (CLL) presented with increasing lymphocytosis and progressive cytopenias. Full blood count revealed hemoglobin 8.9 g/dL, WBC count 13.3 ϫ 10 9 /L, neutrophil count 1.8 ϫ 10 9 /L, lymphocyte count 11.0 ϫ 10 9 /L, and platelets 101 ϫ 10 12 /L. Bone marrow examination (Fig 1A, hematoxylin and eosin [HE] stain) was consistent with disease progression, showing markedly hypercellular bone marrow resulting from interstitial infiltrate of CLL cells coexpressing CD20, CD79a, CD19, CD5, and CD23. Previous treatments included single-agent chlorambucil 7 years before and single-agent fludarabine 5 years before. Therefore, she was administered fludarabine and cyclophosphamide chemotherapy and received her first cycle without complications.After the initial cycle of chemotherapy, the patient was admitted with febrile neutropenia. Broad-spectrum antibiotics were administered. An infection screen, including blood, urine, and sputum cultures and a viral nasal swab, was negative. The patient remained pancytopenic 25 days after chemotherapy, so subcutaneous granulocyte colony-stimulating factor was commenced. On the presumption that the cytopenia represented either ongoing disease or delayed marrow recovery after chemotherapy, a trephine biopsy was performed, which demonstrated markedly hypercellular bone marrow (Fig 1B, HE stain) resulting from a prominent population of CD68ϩ macrophages arranged in sheets (Fig 2A, CD68 stain). There was evidence of phagocytic activity in some areas, consistent with a hemophagocytic syndrome. A minor component of residual CLL infiltration also remained.In light of these findings, additional investigations were performed. Serum ferritin was 12,000 g/L (normal range, 18 to 360 g/L); fibrinogen, 2.3 g/L (normal range, 1.5 to 4.0 g/L); triglycerides, 2.59 mmol/L (normal range, 1.7 to 2.25 mmol/L); and lactate dehy-

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“…Secondary HPS has been sparsely reported in association with CLL. However, most cases were triggered by an infective agent, such as EBV, histoplasmosis, or H1N1 influenza [5, 11–14], by the chemotherapeutic drugs used in the treatment of CLL such as fludarabine or rituximab [4] or by the direct progression of CLL itself [15, 16]. To date there have been no cases in the literature reporting a CLL patient presenting with a secondary HPS due to acquisition of EBV related large cell lymphoma in the setting of profound immunodeficiency as described in this case report.…”

Section: Introductionmentioning

confidence: 99%

Secondary Hemophagocytic Syndrome Associated with Richter’s Transformation in Chronic Lymphocytic Leukemia

El-Haj

Gonsalves

Gupta

et al. 2014

Case Reports in Hematology

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Hemophagocytic syndrome (HPS) is an extremely rare condition arising from the overactivation of one's own immune system. It results in excessive inflammation and tissue destruction. Prompt initiation of treatment is warranted in either scenario in order to decrease mortality. Most cases are triggered by infectious agents, malignancy, or drugs. We describe the first case of a CLL patient presenting with HPS due to acquisition of EBV-related large cell lymphoma in the setting of profound immunodeficiency.

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Histiocytic Medullary Reticulosis Complicating Chronic Lymphocytic Leukaemia: Malignant or Reactive?

Cited by 50 publications

References 19 publications

A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings

A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Chronic Lymphocytic Leukemia after Treatment with Fludarabine, Cyclophosphamide, and Rituximab Chemotherapy, with Autopsy Findings

Hemophagocytic Lymphohistiocytosis in Chronic Lymphocytic Leukemia

Secondary Hemophagocytic Syndrome Associated with Richter’s Transformation in Chronic Lymphocytic Leukemia

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