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  • CLL associated HLH was first documented in the form

    2019-05-15

    CLL associated HLH was first documented in the form of a case series report which included six patients treated for CLL who subsequently developed the phagocytic syndrome months to years later.[7] As these cases occurred prior to publication of the 1991 HLH diagnostic guidelines, the authors postulated that the syndrome was likely reactive to an occult opportunistic viral infection and only subsequently suspected HLH. Subsequent case reports describe CLL associated HLH only in the setting of infection.[8,9] The present report describes “HLH associated CLL” in the absence of an ongoing infectious process. Such an occurrence has only been reported once by Meki et al. [10] In both situations the patients were initially treated per HLH-2004 guidelines.
    Conclusion
    Gelatinous transformation of bone marrow (GTBM) is a rare disorder characterized by loss of adipose and hematopoietic tissues, and deposition of extracellular gelatinous mucopolysaccharides, particularly hyaluronic acid. The common causes of gelatinous transformation of bone marrow are anorexia nervosa, acquired immunodeficiency syndrome, alcoholism, carcinomas, leukemias, lymphomas and chemotherapeutic agents. Imatinib has been reported to cause gelatinous transformation of bone marrow in patients with chronic myeloid leukemia and philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL). We report here a case of Ph+ ALL who was treated with dasatinib, following which he developed gelatinous transformation of bone marrow. An 18-years-old male was admitted with the complaints of generalized weakness and fever for one month duration. On examination he had pallor with cervical lymphadenopathy and hepatosplenomegaly. Hemogram revealed ll-37 5.5g/dl, total leukocyte count 130×10/l, platelet count 40×10/l with peripheral blood smear showing 85% blasts, morphologically lymphoid. Bone marrow examination confirmed the replacement of marrow by lymphoid blasts. On immunophenotyping the blasts were positive for CD34, HLA-DR, CD19, CD20 and cCD79a. Qualitative polymerase chain reaction (PCR) for BCR-ABL was positive. Cerebrospinal fluid examination also revealed the presence of blasts. His viral studies were negative for HBsAg, anti-HCV and HIV. He was started on supportive treatment with intravenous fluids, allopurinol and BFM-95 induction chemotherapy (prednisolone 60mg/m from day 1 and vincristine 1.5mg/m and daunorubicin 30mg/m from day 8) alongwith dasatinib 50mg twice daily from day 1. Intrathecal chemotherapy included methotrexate 12.5mg twice weekly. Patient developed pancytopenia with febrile neutropenia on day 10 and was started on intravenous antibiotics as per institutional policy. Blood and urine cultures were negative for bacteria and fungi. Computed tomography of chest was normal. He was continued on supportive treatment. Peripheral blood smear on day 8 did not show any blasts. Because of persistent fever, amphotericin (1mg/kg) was added along-with granulocyte-colony stimulating factor (G-CSF). Fever continued and patient had persistent cytopenias (total leukocyte count 0.2×10/l, absolute neutrophil count 0.05×10/l and platelet count 10×10/l). Bone marrow examination was repeated on day 32 and it showed serous degeneration of marrow with increased extracellular matrix, loss of fat cells and gelatinous transformation, confirmed with Alcian blue staining (). The overall cellularity of the bone marrow was 5–10%. Normally, gelatinous material is not found in the bone marrow and therefore, its presence signifies a pathological event. Chemotherapeutic drugs, including melphalan and imatinib, have been implicated in the causation of GTBM. There is usually complete recovery of marrow following initial gelatinous transformation in the patients receiving chemotherapy for acute leukemia, but our case showed no marrow recovery. Moreover, GTBM associated with chemotherapy is characterized by absence of fat atrophy and is often transient. Our patient had evidence of fat atrophy along-with gelatinous transformation. GTBM may respond to hematopoietic growth factors but our patient did not have any response to G-CSF and succumbed to febrile neutropenia.